Sample records for akinetic mutism

  1. Akinetic mutism following stroke. (United States)

    Nagaratnam, Nages; Nagaratnam, Kujan; Ng, Kevin; Diu, Patrick


    This is an appraisal of the varied clinical presentation and the neural substrate for akinetic mutism following stroke. The diagnosis is important as akinetic mutism is often misdiagnosed as depression, delirium and locked-in-syndrome. This is a descriptive study of eight selected patients with akinetic mutism following infarction/haemorrhage in different regions of the brain with characteristic syndromes. They involved the critical areas namely, the frontal (cingulate gyrus, supplementary motor area and dorso-lateral border zone), basal ganglia (caudate, putamen), the mesencephalon and thalamus. The disorders of speech and communication took different forms. The speech disorder included verbal inertia, hypophonia, perseveration, softened and at times slurred. The linguistic disturbances were fluent, non-fluent, anomia and transcortical (motor, mixed) aphasias. The findings were related to what is known about the neuroanatomic location of the lesions and the role of the frontal-subcortical circuitry in relation to behaviour. Akinetic mutism could be explained by damage to the frontal lobe and or interruption of the complex frontal subcortical circuits.

  2. Akinetic Mutism Following Bilateral Anterior Cerebral Artery Territory Infarction Due to Aneurysm: A Case Report

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    Zeynep Özözen Ayas


    Full Text Available BACKGROUND AND PURPOSE: Bilateral anterior cerebral artery (ACA territory infarction is rare localization in stroke which should always prompt a search for an anterior communicating artery (ACoA aneurysm. The common neurological manifestations are contralateral weakness predominate in the lower extremite, behavior disturbance, motor inertia, muteness, incontinence, grasp reflex, diffuse rigidity, akinetic mutism. CASE DESCRIPTION: We describe a 38-year-old woman presented with a left sided hemiparesia and decrease of speech for last days. She was a smoker and morbide obese. She had no any diagnosed disease. Her neurological examination had weakness of left extremites affected leg more than the arm and akinetic mutism like as no spontaneously speech and move and grasp reflex. CT showed bilateral ACA infarction which included cingulate gyrus, the right side more than left and subarachnoid hemorrhage in the interhemispheric fissure. MRI angiography showed the appearance of AcoA aneurysm. CONCLUSION: We report a patient with bilateral infarction in the ACA which a rare localization and clinicians must be alert to exist AcoA aneurysm which may bleed, different symptoms and signs like as akinetic mutism, primitive reflexes.

  3. [Investigation of the clinical course and treatment of prion disease patients in the akinetic mutism state in Japan]. (United States)

    Iwasaki, Yasushi; Mori, Keiko; Ito, Masumi


    Twelve cases (one Gerstmann-Sträussler-Scheinker syndrome (P102L; definite), one genetic Creutzfeldt-Jakob disease (CJD) (V180I; definite) and ten sporadic CJD (7 MM1-type definite, 3 probable)), who reached the akinetic mutism state, were investigated with regard to their clinical course and treatment. They were hospitalized for a total of 3,968 days in the akinetic mutism state. In the nine definite cases, the median period from the akinetic mutism state to death was 22 months (average: 27.0 ± 23.3 months, range: 3-80 months) and median total disease duration was 27 months (average: 34.2 ± 30.1 months, range: 5-102 months). In the seven definite sporadic CJD cases, the median period from akinetic mutism to death was 21 months (average: 17.0 ± 9.6 months, range 3-28 months), and median total disease duration was 24 months (average: 20.6 ± 10.0 months, range: 5-31 months). Nasal-tube feeding was performed in all cases. Symptomatic treatments such as parenteral nutrition and antibiotic drugs were administered for complications such as respitory and urinary tract infections and digestive symptoms. Patients received rehabilitation and hot spring therapy regularly until death. Gastrostomy and/or tracheotomy was not performed in any case, the patients were not intubated nor was mechanical ventilation (including non-invasive positive pressure ventilation) applied. Vasoactive drugs were not administered. Clonazepam was administered for myoclonus in four patients but not in another three when myoclonus appeared. It is unclear whether the treatment influenced the duration of myoclonus. Our observations indicate that the extended survival period among Japanese prion disease patients is likely due to the management procedures implemented for prion disease in Japan, which are usually continued after the patients reach the akinetic mutism state. We speculate that nasal-tube feeding is the crucial factor that results in the prolonged disease duration of prion disease

  4. Reversible Akinetic Mutism after Aneurysmal Subarachnoid Haemorrhage in the Territory of the Anterior Cerebral Artery without Permanent Ischaemic Damage to Anterior Cingulate Gyri

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    François-Xavier Sibille


    Full Text Available We report on two cases of transient akinetic mutism after massive subarachnoid haemorrhage due to the rupture of an intracranial aneurysm of the anterior cerebral artery (ACA. In the two cases, vasospasm could not be demonstrated by imaging studies throughout the clinical course. Both patients shared common radiological features: a hydrocephalus due to haemorrhagic contamination of the ventricular system and a mass effect of a subpial hematoma on the borders of the corpus callosum. Patients were also investigated using auditory event-related evoked potentials at acute stage. In contrast to previous observations of akinetic mutism, P300 wave could not be recorded. Both patients had good recovery and we hypothesized that this unexpectedly favourable outcome was due to the absence of permanent structural damage to the ACA territory, with only transient dysfunction due to a reversible mass effect on cingulate gyri.

  5. Dysarthria and mutism. (United States)

    Sternic, Nadezda; Mijajlovic, Milija; Tomic, Gordana; Pavlovic, Aleksandra


    Dysarthria is a speech disorder associated with impairments of intelligibility, smoothness, loudness, and clarity of articulations. Dysarthria involves disability of reproducing various physical, tonal, and sound features of speech sounds in oral speech; unintelligible and slurred articulation with swallowing of sounds is characteristic. Articulatory movements and speech are slow, patients complain to the sensations of a 'thick' tongue and 'porridge' in the mouth. Patients'phrases are constructed correctly, vocabulary is not affected, and the grammatical structure of words is preserved. Reading, writing, internal speech, and understanding of speech are unaffected. Several types of dysarthria have been described on the basis of the lesion locations. Dysarthria can be associated with lacunar syndromes as well. Mutism represents a condition when patient cannot speak and answer the questions, but remains conscious and is able to produce written speech.

  6. Mesoproterozoic Archaeoellipsoides: akinetes of heterocystous cyanobacteria (United States)

    Golubic, S.; Sergeev, V. N.; Knoll, A. H.


    The genus Archaeoellipsoides Horodyski & Donaldson comprises large (up to 135 micrometers long) ellipsoidal and rod-shaped microfossils commonly found in silicified peritidal carbonates of Mesoproterozoic age. Based on morphometric and sedimentary comparisons with the akinetes of modern bloom-forming Anabaena species, Archaeoellipsoides is interpreted as the fossilized remains of akinetes produced by planktic heterocystous cyanobacteria. These fossils set a minimum date for the evolution of derived cyanobacteria capable of marked cell differentiation, and they corroborate geochemical evidence indicating that atmospheric oxygen levels were well above 1% of present day levels 1,500 million years ago.

  7. [Bromocriptine: could it be the cure for post-surgical akinetic mutism? (United States)

    Arevalo-Saenz, A; Pedrosa-Sanchez, M; Sola, R G


    Introduccion. El mutismo acinetico se considera una alteracion del estado motivacional de la persona, por el cual el paciente es incapaz de iniciar respuestas verbales o motoras de caracter voluntario, aun teniendo preservadas las funciones sensomotoras y de vigilancia. Caso clinico. Varon de 43 años, intervenido de una fistula arteriovenosa del cerebelo complicada con hidrocefalia, que respondio espectacularmente al tratamiento con bromocriptina. Conclusion. Tipicamente se ha descrito el mutismo acinetico como una complicacion transitoria de las cirugias de la fosa posterior. Sin embargo, tambien puede aparecer tras multiples fallos valvulares en pacientes con hidrocefalia.

  8. Deciphering the mechanisms against oxidative stress in developing and mature akinetes of the cyanobacterium Aphanizomenon ovalisporum. (United States)

    Kaplan-Levy, Ruth N; Hadas, Ora; Sukenik, Assaf


    Cells of filamentous cyanobacteria of the orders Nostocales and Stigonematales can differentiate into dormant forms called akinetes. Akinetes play a key role in the survival, abundance and distribution of the species, contributing an inoculum for their perennial blooms. In the cyanobacterium Aphanizomenon ovalisporum, potassium deficiency triggers the formation of akinetes. Here we present experimental evidence for the production of reactive oxygen species (ROS) during akinete development in response to potassium deficiency. The function of ROS as a primer signal for akinete differentiation was negated. Nevertheless, akinetes acquired protective mechanisms against oxidative damage during their differentiation and maintained them as they matured, giving akinetes advantages enabling survival in harsh conditions.

  9. Cerebellar mutism: review of the literature

    DEFF Research Database (Denmark)

    Gudrunardottir, Thora; Sehested, Astrid; Juhler, Marianne;


    Cerebellar mutism is a common complication of posterior fossa surgery in children. This article reviews current status with respect to incidence, anatomical substrate, pathophysiology, risk factors, surgical considerations, treatment options, prognosis and prevention.......Cerebellar mutism is a common complication of posterior fossa surgery in children. This article reviews current status with respect to incidence, anatomical substrate, pathophysiology, risk factors, surgical considerations, treatment options, prognosis and prevention....

  10. Selective Mutism: Treating the Silent Child (United States)

    Shott, Elizabeth F.; Warren, Mary Ellen


    Infant mental health specialists are increasingly expected to treat complex mental health disorders in very young children. Selective mutism is an anxiety disorder which can lead to functional impairment across home, preschool, and community settings. The authors share their experiences with Keylah, a preschooler with significant social anxiety…

  11. Potassium deficiency triggers the development of dormant cells (akinetes) in Aphanizomenon ovalisporum (Nostocales, Cyanoprokaryota)(1). (United States)

    Sukenik, Assaf; Kaplan-Levy, Ruth N; Viner-Mozzini, Yehudit; Quesada, Antonio; Hadas, Ora


    Akinetes are spore-like nonmotile cells that differentiate from vegetative cells of filamentous cyanobacteria from the order Nostocales. They play a key role in the survival and distribution of these species and contribute to their perennial blooms. Various environmental factors were reported to trigger the differentiation of akinetes including light intensity and quality, temperature, and nutrient deficiency. Here, we report that deprivation of potassium ion (K(+) ) triggers akinete development in the cyanobacterium Aphanizomenon ovalisporum. Akinetes formation is initiated 3 d-7 d after an induction by K(+) depletion, followed by 2-3 weeks of a maturation process. Akinete formation occurs within a restricted matrix of environmental conditions such as temperature, light intensity or photon flux. Phosphate is essential for akinete maturation and P-limitation restricts the number of mature akinetes. DNA replication is essential for akinete maturation and akinete development is limited in the presence of Nalidixic acid. While our results unequivocally demonstrated the effect of K(+) deficiency on akinete formation in laboratory cultures of A. ovalisporum, this trigger did not cause Cylindrospermopsis raciborskii to produce akinetes. Anabaena crassa however, produced akinetes upon potassium deficiency, but the highest akinete concentration was achieved at conditions that supported vegetative growth. It is speculated that an unknown internal signal is associated with the cellular response to K(+) deficiency to induce the differentiation of a certain vegetative cell in a trichome into an akinete. A universal stress protein that functions as mediator in K(+) deficiency signal transduction cascade, may communicate between the lack of K(+) and akinete induction.

  12. Selective Serotonin Reuptake Inhibitors for Treatment of Selective Mutism

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    Mazlum Çöpür


    Full Text Available Some authors suggest that selective mutism should be considered as a variant of social phobia or a disorder in the obsessive-compulsive spectrum. Recent studies indicate that pharmacological treatments may be effective in the treatment of selective mutism. In this article, four cases who were treated with citalopram and escitalopram are presented. The results indicate that the drugs were well tolerated, and the level of social and verbal interactions improved significantly. These findings have shown that citalopram and escitalopram can be considered in medication of selective mutism; nevertheless, it is essential that research be done with more cases than previous ones, in order to prove their accuracy

  13. Massive multiplication of genome and ribosomes in dormant cells (akinetes) of Aphanizomenon ovalisporum (Cyanobacteria). (United States)

    Sukenik, Assaf; Kaplan-Levy, Ruth N; Welch, Jessica Mark; Post, Anton F


    Akinetes are dormancy cells commonly found among filamentous cyanobacteria, many of which are toxic and/or nuisance, bloom-forming species. Development of akinetes from vegetative cells is a process that involves morphological and biochemical modifications. Here, we applied a single-cell approach to quantify genome and ribosome content of akinetes and vegetative cells in Aphanizomenon ovalisporum (Cyanobacteria). Vegetative cells of A. ovalisporum were naturally polyploid and contained, on average, eight genome copies per cell. However, the chromosomal content of akinetes increased up to 450 copies, with an average value of 119 genome copies per akinete, 15-fold higher than that in vegetative cells. On the basis of fluorescence in situ hybridization, with a probe targeting 16S rRNA, and detection with confocal laser scanning microscopy, we conclude that ribosomes accumulated in akinetes to a higher level than that found in vegetative cells. We further present evidence that this massive accumulation of nucleic acids in akinetes is likely supported by phosphate supplied from inorganic polyphosphate bodies that were abundantly present in vegetative cells, but notably absent from akinetes. These results are interpreted in the context of cellular investments for proliferation following a long-term dormancy, as the high nucleic acid content would provide the basis for extended survival, rapid resumption of metabolic activity and cell division upon germination.

  14. Nonsurgical cerebellar mutism (anarthria) in two children. (United States)

    Mewasingh, Leena D; Kadhim, Hazim; Christophe, Catherine; Christiaens, Florence J; Dan, Bernard


    Cerebellar mutism (anarthria) is a well-described complication of posterior fossa tumor resection. It is accompanied by a characteristic behavior including irritability and autistic features. This syndrome is typically reversible within days to months. Underlying pathophysiology is unknown. We describe two children who presented with a similar clinical finding after nonsurgical cerebellar involvement, hemolytic-uremic syndrome in one and cerebellitis in the other. Postmortem pathologic findings in the first patient indicated cerebellar ischemic necrosis. Single-photon emission computed tomography in the second patient revealed diffuse cerebellar hypoperfusion with no supratentorial abnormalities, refuting a phenomenon of diaschisis between cerebellar and frontal connections. These findings confirm that this clinical syndrome may occur in a nonsurgical, nontraumatic context. They are consistent with recent integrative hypotheses explaining cerebellar anarthria.

  15. A CARD-FISH protocol for the identification and enumeration of cyanobacterial akinetes in lake sediments. (United States)

    Ramm, Jessica; Lupu, Achsa; Hadas, Ora; Ballot, Andreas; Rücker, Jacqueline; Wiedner, Claudia; Sukenik, Assaf


    Akinetes are the dormant cells of Nostocales (cyanobacteria) that enable the organisms to survive harsh environmental conditions while resting in bottom sediments. The germination of akinetes assists the dispersal and persistence of the species. The assessment of the akinete pool in lake sediments is essential to predict the bloom formation of the Nostocales population. We present here the implementation of an improved catalysed reporter deposition (CARD)-fluorescence in situ hybridization (FISH) protocol to assist the identification and quantification of akinetes in sediment samples. Several 16S rRNA gene oligonucleotide probes were evaluated for labelling akinetes of various species of Anabaena, Aphanizomenon and Cylindrospermopsis. Akinetes of all the taxa studied were successfully labelled and could be easily detected by their bright fluorescence signal. The probes' specificity was tested with 32 strains of different taxa. All six Cylindrospermopsis raciborskii strains were labelled with a specific probe for its 16S rRNA gene. A more general probe labelled 73% of the Anabaena and Aphanizomenon strains. The counting data of field samples obtained with CARD-FISH and the regular light microscopy approach did not differ significantly, confirming the suitability of both methods. The CARD-FISH approach was found to be less time-consuming because of better visibility of akinetes.

  16. Assessment and Treatment of Selective Mutism with English Language Learners (United States)

    Mayworm, Ashley M.; Dowdy, Erin; Knights, Kezia; Rebelez, Jennica


    Selective mutism (SM) is a type of anxiety disorder that involves the persistent failure to speak in contexts where speech is typically expected (e.g., school), despite speaking in other contexts (e.g., home). Research on the etiology and treatment of SM is limited, as it is a rare disorder and few clinical trials evaluating SM interventions have…

  17. Selective Mutism: Practice and Intervention Strategies for Children (United States)

    Hung, Shu-Lan; Spencer, Michael S.; Dronamraju, Rani


    The onset of selective mutism (SM) is usually between the ages of three and five years, when the children first go to preschool. However, these children are most commonly referred for treatment between the ages of six and 11, when they are entering the elementary school system. Early detection and early intervention is suggested for effective SM…

  18. Survival of akinetes (resting-state cells of cyanobacteria) in low earth orbit and simulated extraterrestrial conditions. (United States)

    Olsson-Francis, Karen; de la Torre, Rosa; Towner, Martin C; Cockell, Charles S


    Cyanobacteria are photosynthetic organisms that have been considered for space applications, such as oxygen production in bioregenerative life support systems, and can be used as a model organism for understanding microbial survival in space. Akinetes are resting-state cells of cyanobacteria that are produced by certain genera of heterocystous cyanobacteria to survive extreme environmental conditions. Although they are similar in nature to endospores, there have been no investigations into the survival of akinetes in extraterrestrial environments. The aim of this work was to examine the survival of akinetes from Anabaena cylindrica in simulated extraterrestrial conditions and in Low Earth Orbit (LEO). Akinetes were dried onto limestone rocks and sent into LEO for 10 days on the ESA Biopan VI. In ground-based experiments, the rocks were exposed to periods of desiccation, vacuum (0.7×10(-3) kPa), temperature extremes (-80 to 80°C), Mars conditions (-27°C, 0.8 kPa, CO(2)) and UV radiation (325-400 nm). A proportion of the akinete population was able to survive a period of 10 days in LEO and 28 days in Mars simulated conditions, when the rocks were not subjected to UV radiation. Furthermore, the akinetes were able to survive 28 days of exposure to desiccation and low temperature with high viability remaining. Yet long periods of vacuum and high temperature were lethal to the akinetes. This work shows that akinetes are extreme-tolerating states of cyanobacteria that have a practical use in space applications and yield new insight into the survival of microbial resting-state cells in space conditions.

  19. Selective mutism and temperament: the silence and behavioral inhibition to the unfamiliar. (United States)

    Gensthaler, Angelika; Khalaf, Sally; Ligges, Marc; Kaess, Michael; Freitag, Christine M; Schwenck, Christina


    Behavioral inhibition (BI) is a suspected precursor of selective mutism. However, investigations on early behavioral inhibition of children with selective mutism are lacking. Children aged 3-18 with lifetime selective mutism (n = 109), social phobia (n = 61), internalizing behavior (n = 46) and healthy controls (n = 118) were assessed using the parent-rated Retrospective Infant Behavioral Inhibition (RIBI) questionnaire. Analyses showed that children with lifetime selective mutism and social phobia were more inhibited as infants and toddlers than children of the internalizing and healthy control groups, who displayed similar low levels of behavioral inhibition. Moreover, behavioral inhibition was higher in infants with lifetime selective mutism than in participants with social phobia according to the Total BI score (p = 0.012) and the Shyness subscale (p lifetime diagnosis of selective mutism. Results yield first evidence of the recently hypothesized temperamental origin of selective mutism. Children at risk should be screened for this debilitating child psychiatric condition.

  20. Consensus paper on post-operative pediatric cerebellar mutism syndrome

    DEFF Research Database (Denmark)

    Gudrunardottir, Thora; Morgan, Angela T; Lux, Andrew L


    INTRODUCTION: Confusion has surrounded the description of post-operative mutism and associated morbidity in pediatric patients with cerebellar tumors for years. The heterogeneity of definitions and diagnostic features has hampered research progress within the field, and to date, no international...... guidelines exist on diagnosis, prevention, treatment, or follow-up of this debilitating condition. An international group of clinicians and researchers from multiple relevant disciplines recently formed a cohesive panel to formulate a new working definition and agree upon standardized methods for diagnosis...... and follow-up. METHODS: Consensus was obtained using the modified nominal group technique, involving four rounds of online Delphi questionnaires interspersed with a structured consensus conference with lectures, group work, and open discussion sessions. RESULTS: A new, proposed definition of "post-operative...

  1. Comorbidity and Family Factors Associated with Selective Mutism

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    Brian A. Buzzella


    Full Text Available Recent findings suggest that Selective Mutism (SM is best conceptualized as a childhood anxiety disorder and that oppositional behavior may or may not be a significant part of the clinical picture. Twenty-nine mothers of children with SM and 28 mothers of children who did not meet diagnostic criteria for any Axis I disorder (a community comparison group completed parental self-report questionnaires and clinician-rated interviews assessing anxiety and oppositional behavior, parental psychopathology, and family factors with hypothesized relationships with childhood anxiety. Findings suggested that children with SM experienced more anxiety than those in the community comparison group, with significantly higher levels of social anxiety, rumination, and physical symptoms reported. Mothers of children with SM reported greater monitoring of their children's activities, but they did not significantly differ from community comparison group mothers on reports of other parenting behaviors. Such findings may have important implications for guiding family involvement in psychosocial interventions.

  2. Tractography demonstrates dentate-rubro-thalamic tract disruption in an adult with cerebellar mutism

    NARCIS (Netherlands)

    Baarsen, van K.; Kleinnijenhuis, M.; Konert, T.; Cappellen van Walsum, A.; Grotenhuis, A.


    A 55-year-old female is presented with transient cerebellar mutism caused by a well-circumscribed left pontine infarction due to postoperative basilar perforator occlusion. Although conventional T2 imaging shows a well-demarcated lesion confined to the pontine region, diffusion tensor imaging shows

  3. Selective Mutism: A Team Approach to Assessment and Treatment in the School Setting (United States)

    Ponzurick, Joan M.


    The school nurse plays a pivotal role in the assessment and treatment of selective mutism (SM), a rare disorder found in elementary school children. Due to anxiety, children with SM do not speak in uncomfortable situations, primarily the school setting. Diagnosis of SM is often missed in the formative years because the child does speak at home.…

  4. Assessing Spoken Language Competence in Children with Selective Mutism: Using Parents as Test Presenters (United States)

    Klein, Evelyn R.; Armstrong, Sharon Lee; Shipon-Blum, Elisa


    Children with selective mutism (SM) display a failure to speak in select situations despite speaking when comfortable. The purpose of this study was to obtain valid assessments of receptive and expressive language in 33 children (ages 5 to 12) with SM. Because some children with SM will speak to parents but not a professional, another purpose was…

  5. Examination of a Social Problem-Solving Intervention to Treat Selective Mutism

    NARCIS (Netherlands)

    O'Reilly, M.F.; McNally, D.; Sigafoos, J.; Lancioni, G.E.; Green, V.A.; Edrisinha, C.; Machalicek, W.A.; Sorrells, A.; Didden, H.C.M.


    The authors examined the use of a social problem-solving intervention to treat selective mutism with 2 sisters in an elementary school setting. Both girls were taught to answer teacher questions in front of their classroom peers during regular classroom instruction. Each girl received individualized

  6. A Long-Term Outcome Study of Selective Mutism in Childhood (United States)

    Steinhausen, Hans-Christoph; Wachter, Miriam; Laimbock, Karin; Metzke, Christa Winkler


    Objective: Controlled study of the long-term outcome of selective mutism (SM) in childhood. Method: A sample of 33 young adults with SM in childhood and two age- and gender-matched comparison groups were studied. The latter comprised 26 young adults with anxiety disorders in childhood (ANX) and 30 young adults with no psychiatric disorders during…

  7. The Current State of Empirical Support for the Pharmacological Treatment of Selective Mutism (United States)

    Carlson, John S.; Mitchell, Angela D.; Segool, Natasha


    This article reviews the current state of evidence for the psychopharmacological treatment of children diagnosed with selective mutism within the context of its link to social anxiety disorder. An increased focus on potential medication treatment for this disorder has resulted from significant monetary and resource limitations in typical practice,…

  8. Evaluation of Children with Selective Mutism and Social Phobia: A Comparison of Psychological and Psychophysiological Arousal (United States)

    Young, Brennan J.; Bunnell, Brian E.; Beidel, Deborah C.


    Although children with social phobia (SP) and selective mutism (SM) present similarly in a clinical setting, it remains unclear whether children with SM are unable to speak due to overwhelming anxiety, or whether withholding speech functions as an avoidance mechanism. A total of 35 children (ages 5-12 years) with either SM (n = 10), SP (n = 11),…

  9. Contributions to the functional morphology of caudate skulls: kinetic and akinetic forms. (United States)

    Natchev, Nikolay; Handschuh, Stephan; Lukanov, Simeon; Tzankov, Nikolay; Naumov, Borislav; Werneburg, Ingmar


    A strongly ossified and rigid skull roof, which prevents parietal kinesis, has been reported for the adults of all amphibian clades. Our μ-CT investigations revealed that the Buresch's newt (Triturus ivanbureschi) possess a peculiar cranial construction. In addition to the typical amphibian pleurokinetic articulation between skull roof and palatoquadrate associated structures, we found flexible connections between nasals and frontals (prokinesis), vomer and parasphenoid (palatokinesis), and between frontals and parietals (mesokinesis). This is the first description of mesokinesis in urodelans. The construction of the skull in the Buresch's newts also indicates the presence of an articulation between parietals and the exocipitals, discussed as a possible kind of metakinesis. The specific combination of pleuro-, pro-, meso-, palato-, and metakinetic skull articulations indicate to a new kind of kinetic systems unknown for urodelans to this date. We discuss the possible neotenic origin of the skull kinesis and pose the hypothesis that the kinesis in T. ivanbureschi increases the efficiency of fast jaw closure. For that, we compared the construction of the skull in T. ivanbureschi to the akinetic skull of the Common fire salamander Salamandra salamandra. We hypothesize that the design of the skull in the purely terrestrial living salamander shows a similar degree of intracranial mobility. However, this mobility is permitted by elasticity of some bones and not by true articulation between them. We comment on the possible relation between the skull construction and the form of prey shaking mechanism that the species apply to immobilize their victims.

  10. Contributions to the functional morphology of caudate skulls: kinetic and akinetic forms

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    Nikolay Natchev


    Full Text Available A strongly ossified and rigid skull roof, which prevents parietal kinesis, has been reported for the adults of all amphibian clades. Our μ-CT investigations revealed that the Buresch’s newt (Triturus ivanbureschi possess a peculiar cranial construction. In addition to the typical amphibian pleurokinetic articulation between skull roof and palatoquadrate associated structures, we found flexible connections between nasals and frontals (prokinesis, vomer and parasphenoid (palatokinesis, and between frontals and parietals (mesokinesis. This is the first description of mesokinesis in urodelans. The construction of the skull in the Buresch’s newts also indicates the presence of an articulation between parietals and the exocipitals, discussed as a possible kind of metakinesis. The specific combination of pleuro-, pro-, meso-, palato-, and metakinetic skull articulations indicate to a new kind of kinetic systems unknown for urodelans to this date. We discuss the possible neotenic origin of the skull kinesis and pose the hypothesis that the kinesis in T. ivanbureschi increases the efficiency of fast jaw closure. For that, we compared the construction of the skull in T. ivanbureschi to the akinetic skull of the Common fire salamander Salamandra salamandra. We hypothesize that the design of the skull in the purely terrestrial living salamander shows a similar degree of intracranial mobility. However, this mobility is permitted by elasticity of some bones and not by true articulation between them. We comment on the possible relation between the skull construction and the form of prey shaking mechanism that the species apply to immobilize their victims.

  11. Orbitofrontal cortical dysfunction in akinetic catatonia: a functional magnetic resonance imaging study during negative emotional stimulation. (United States)

    Northoff, Georg; Kötter, Rolf; Baumgart, Frank; Danos, Peter; Boeker, Heinz; Kaulisch, Thomas; Schlagenhauf, Florian; Walter, Henrik; Heinzel, Alexander; Witzel, Thomas; Bogerts, Bernhard


    Catatonia is a psychomotor syndrome characterized by concurrent emotional, behavioral, and motor anomalies. Pathophysiological mechanisms of psychomotor disturbances may be related to abnormal emotional-motor processing in prefrontal cortical networks. We therefore investigated prefrontal cortical activation and connectivity patterns during emotional-motor stimulation using functional magnetic resonance imaging (FMRI). We investigated 10 akinetic catatonic patients in a postacute state and compared them with 10 noncatatonic postacute psychiatric controls (age-, sex-, diagnosis-, and medication-matched) and 10 healthy controls. Positive and negative pictures from the International Affective Picture System were used for emotional stimulation. FMRI measurements covered the whole frontal lobe, activation signals in various frontal cortical regions were obtained, and functional connectivity between the different prefrontal cortical regions was investigated using structural equation modeling. Catatonic patients showed alterations in the orbitofrontal cortical activation pattern and in functional connectivity to the premotor cortex in negative and positive emotions compared to psychiatric and healthy controls. Catatonic behavioral and affective symptoms correlated significantly with orbitofrontal activity, whereas catatonic motor symptoms were rather related to medial prefrontal activity. It is concluded that catatonic symptoms may be closely related to dysfunction in the orbitofrontal cortex and consequent alteration in the prefrontal cortical network during emotional processing. Because we investigated postacute patients, orbitofrontal cortical alterations may be interpreted as a trait marker predisposing for development of catatonic syndrome in schizophrenic or affective psychosis.

  12. Rapid response of long-standing, treatment-resistant non-catatonic mutism in paranoid schizophrenia with single ECT session

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    Mansoor Ahmad Dar


    Full Text Available Context: Mutism is a common manifestation of catatonia, but mutism due to other forms of psychopathology and neurological disorders have also been described. Although not common, long-standing mutism has also been a feature of non-catatonic schizophrenia and traditionally responds less to conventional therapies. Case Report: We describe a rare case of paranoid schizophrenia presenting with continuous mutism for about 4 years. This 26-year-old male had symptoms of schizophrenia without catatonia. After failed trial of adequate pharmacotherapy and psychological intervention and considering his level of dysfunction, he was started on electroconvulsive therapy (ECT. To our surprise, he improved with a single session of ECT while he was on concurrent pharmacotherapy. We also discuss the possible explanation for this rapid effect of ECT in such clinical presentation. To our knowledge, this is the first case of non-catatonic mutism of schizophrenia of this long duration responding so promptly to ECT, although there are other reports as well in literature, but multiple ECT sessions were applied in those cases. Conclusion: Non-catatonic mutism is perhaps presenting as a cultural variant in this part of the world and whenever encountered, ECT should be an option. Further research should be carried out to validate this idea.

  13. [Multilingualism and child psychiatry: on differential diagnoses of language disorder, specific learning disorder, and selective mutism]. (United States)

    Tamiya, Satoshi


    Multilingualism poses unique psychiatric problems, especially in the field of child psychiatry. The author discusses several linguistic and transcultural issues in relation to Language Disorder, Specific Learning Disorder and Selective Mutism. Linguistic characteristics of multiple language development, including so-called profile effects and code-switching, need to be understood for differential diagnosis. It is also emphasized that Language Disorder in a bilingual person is not different or worse than that in a monolingual person. Second language proficiency, cultural background and transfer from the first language all need to be considered in an evaluation for Specific Learning Disorder. Selective Mutism has to be differentiated from the silent period observed in the normal successive bilingual development. The author concludes the review by remarking on some caveats around methods of language evaluation in a multilingual person.

  14. A Selective Mutism Arising from First Language Attrition, Successfully Treated with Paroxetine-CBT Combination Treatment. (United States)

    Serra, Agostino; Di Mauro, Paola; Andaloro, Claudio; Maiolino, Luigi; Pavone, Piero; Cocuzza, Salvatore


    After immersion in a foreign language, speakers often have difficulty retrieving native-language words and may experience a decrease in its proficiency, this phenomenon, in the non-pathological form, is known as first language attrition. Self-perception of this low native-language proficiency and apprehension occurring when speaking is expected and, may sometimes lead these people to a state of social anxiety and, in extreme forms, can involve the withholding of speech as a primitive tool for self-protection, linking them to selective mutism. We report an unusual case of selective mutism arising from first language attrition in an Italian girl after attending a two-year "German language school", who successfully responded to a paroxetine-cognitive behavioral treatment (CBT) combination treatment.

  15. Akinetic Crisis in Parkinson's Disease Is Associated with a Severe Loss of Striatal Dopamine Transporter Function: A Report of Two Cases

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    Valtteri Kaasinen


    Full Text Available Akinetic crisis or acute akinesia is a life-threatening complication of Parkinson's disease (PD with unknown pathophysiological mechanisms. Clinically, it resembles the neuroleptic malignant syndrome, and dopaminergic drugs are transiently ineffective in the acute phase of the condition. There are no published dopaminergic functional imaging studies on PD patients with akinetic crisis. Here we report 2 advanced PD patients with akinetic crisis who were scanned with SPECT using brain dopamine transporter ligand [123I]FP-CIT. The first patient was additionally scanned before the condition developed, and the second patient was scanned after recovery. Striatal dopamine transporter binding was lower during than before the crisis, and both patients showed a nearly complete loss of dopamine transporter binding during the crisis. Serial imaging showed that the uptake remained negligible despite an improvement in motor function after recovery. Akinetic crisis in PD appears to be associated with a particularly severe loss of presynaptic striatal dopamine function that does not improve after recovery. Apart from presynaptic dopaminergic function, other dopaminergic or nondopaminergic mechanisms are involved in the clinical improvement of motor functions after akinetic crisis in PD.

  16. Phase-sensitive optical coherence tomography-based vibrometry using a highly phase-stable akinetic swept laser source

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    Applegate, Brian E.; Park, Jesung; Carbajal, Esteban [Department of Biomedical Engineering, Texas A& M University, College Station, Texas (United States); Oghalai, John S. [Department of Otolaryngology - Head and Neck Surgery, Stanford University, Stanford, California (United States)


    Phase-sensitive Optical Coherence Tomography (PhOCT) is an emerging tool for in vivo investigation of the vibratory function of the intact middle and inner ear. PhOCT is able to resolve micron scale tissue morphology in three dimensions as well as measure picometer scale motion at each spatial position. Most PhOCT systems to date have relied upon the phase stability offered by spectrometer detection. On the other hand swept laser source based PhOCT offers a number of advantages including balanced detection, long imaging depths, and high imaging speeds. Unfortunately the inherent phase instability of traditional swept laser sources has necessitated complex user developed hardware/software solutions to restore phase sensitivity. Here we present recent results using a prototype swept laser that overcomes these issues. The akinetic swept laser is electronically tuned and precisely controls sweeps without any mechanical movement, which results in high phase stability. We have developed an optical fiber based PhOCT system around the akinetic laser source that had a 1550 nm center wavelength and a sweep rate of 140 kHz. The stability of the system was measured to be 4.4 pm with a calibrated reflector, thus demonstrating near shot noise limited performance. Using this PhOCT system, we have acquired structural and vibratory measurements of the middle ear in a mouse model, post mortem. The quality of the results suggest that the akinetic laser source is a superior laser source for PhOCT with many advantages that greatly reduces the required complexity of the imaging system.

  17. Switching to aripiprazole for the treatment of residual mutism resulted in distinct clinical courses in two catatonic schizophrenia cases (United States)

    Muneoka, Katsumasa; Kanahara, Nobuhisa; Kimura, Shou


    Objectives: The efficacy of a partial agonist for the dopamine D2 receptor, aripiprazole, for catatonia in schizophrenia has been reported. Methods: We report distinct clinical courses in challenging aripiprazole to treat residual mutism after severe catatonic symptoms improved. Results: In the first case, mutism was successfully treated when the patient was switched from olanzapine to aripiprazole. In contract, switching to aripiprazole from risperidone aggravated auditory hallucinations in the second case. Conclusions: We will discuss the benefits and risks of using aripiprazole for the treatment of catatonic schizophrenia and the possibility of dopamine supersensitivity psychosis. PMID:28255444

  18. An auditory-neuroscience perspective on the development of selective mutism

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    Yael Henkin


    Full Text Available Selective mutism (SM is a relatively rare psychiatric disorder of childhood characterized by consistent inability to speak in specific social situations despite the ability to speak normally in others. SM typically involves severe impairments in social and academic functioning. Common complications include school failure, social difficulties in the peer group, and aggravated intra-familial relationships. Although SM has been described in the medical and psychological literatures for many years, the potential underlying neural basis of the disorder has only recently been explored. Here we explore the potential role of specific auditory neural mechanisms in the psychopathology of SM and discuss possible implications for treatment.

  19. Postoperative motor speech production in children with the syndrome of 'cerebellar' mutism and subsequent dysarthria: a critical review of the literature. (United States)

    De Smet, Hyo Jung; Baillieux, Hanne; Catsman-Berrevoets, Coriene; De Deyn, Peter P; Mariën, Peter; Paquier, Philippe F


    Transient cerebellar mutism is a well-known clinical entity which may develop after surgery to the cerebellum. As the period of mutism is followed by motor speech deficits, the condition has also been termed the syndrome of (cerebellar) Mutism and Subsequent Dysarthria (MSD). In children, its incidence is estimated between 8% and 31%. Unfortunately, the literature provides contradictory information regarding motor speech production post-mutism. We therefore critically reviewed data on 283 childhood cases to chart the mode of recovery of motor speech production after the mute period. After applying stringent exclusion criteria, we found that 98.8% of the children displayed motor speech deficits. This percentage is much higher than commonly reported in the literature. In addition, recovery of speech appeared to be less favourable than previously ascertained. Future studies should investigate more carefully the patients' speech characteristics in order to be able to offer children an adequate and complete rehabilitation program.

  20. Cerebellar mutism caused by primary varicella infection in an immunocompetent child. (United States)

    Erol, Ilknur; Özkale, Yasemin; Saygi, Semra; Alehan, Füsun


    Varicella (chickenpox) is a common childhood infection caused by the varicella-zoster virus, which is often self-limiting and usually benign. Although uncommon, neurologic complications of varicella have been documented that include postinfectious cerebellar ataxia, meningoencephalitis, Reye syndrome, myelitis, optic neuritis, stroke, Guillain-Barré syndrome, seventh cranial nerve palsy, and Ramsay-Hunt syndrome. In this case study, the authors describe a 7-year-old girl who presented with varicella skin rash with unsteady gait and anarthria on day 2, and her condition was attributed to cerebellar mutism. To date, this complication has never been reported in a child with primary varicella infection. Therefore, this case study documents a rare but serious complication of childhood chickenpox.

  1. Cerebellar Mutism



    Of a series of 15 children operated for cerebellar tumor at University Hospital Rotterdam-Dijkzigt, The Netherlands, 5 developed “cerebellar mutism” and subsequent dysarthria after surgery, and 2 had mild speech problems.

  2. Selective Mutism (United States)

    ... the 2013 edition of the Diagnostic and Statistical Manual of Mental Disorders: Fifth Edition (DSM-5: pp. ... reports parent/teacher comments previous testing (e.g., psychological) standardized testing The hearing screening seeks information on: ...

  3. Dopamine transporter single-photon emission computerized tomography supports diagnosis of akinetic crisis of parkinsonism and of neuroleptic malignant syndrome. (United States)

    Martino, G; Capasso, M; Nasuti, M; Bonanni, L; Onofrj, M; Thomas, A


    Akinetic crisis (AC) is akin to neuroleptic malignant syndrome (NMS) and is the most severe and possibly lethal complication of parkinsonism. Diagnosis is today based only on clinical assessments yet is often marred by concomitant precipitating factors. Our purpose is to evidence that AC and NMS can be reliably evidenced by FP/CIT single-photon emission computerized tomography (SPECT) performed during the crisis. Prospective cohort evaluation in 6 patients. In 5 patients, affected by Parkinson disease or Lewy body dementia, the crisis was categorized as AC. One was diagnosed as having NMS because of exposure to risperidone. In all FP/CIT, SPECT was performed in the acute phase. SPECT was repeated 3 to 6 months after the acute event in 5 patients. Visual assessments and semiquantitative evaluations of binding potentials (BPs) were used. To exclude the interference of emergency treatments, FP/CIT BP was also evaluated in 4 patients currently treated with apomorphine. During AC or NMS, BP values in caudate and putamen were reduced by 95% to 80%, to noise level with a nearly complete loss of striatum dopamine transporter-binding, corresponding to the "burst striatum" pattern. The follow-up re-evaluation in surviving patients showed a recovery of values to the range expected for Parkinsonisms of same disease duration. No binding effects of apomorphine were observed. By showing the outstanding binding reduction, presynaptic dopamine transporter ligand can provide instrumental evidence of AC in Parkinsonism and NMS.

  4. Social Communication Anxiety Treatment (S-CAT) for children and families with selective mutism: A pilot study. (United States)

    Klein, Evelyn R; Armstrong, Sharon Lee; Skira, Kathryn; Gordon, Janice


    This research assessed the feasibility of Social Communication Anxiety Treatment (S-CAT) developed by Elisa Shipon-Blum, a brief multimodal approach, to increase social communication in 40 children aged 5-12 years with selective mutism (SM). SM is a disorder in which children consistently fail to speak in specific situations although they have the ability to do so. Key features of this approach are the SM-Social Communication Comfort Scale (SCCS), transfer of control (ToC), a nonchalant therapeutic style, and cognitive-behavioral strategies over a brief time frame. Following 9 weeks of treatment, children showed significant gains in speaking frequency on all 17 items from the Selective Mutism Questionnaire (SMQ), a standardized measure of SM severity. Children also showed decreased levels of anxiety and withdrawal as reported by parents on the Child Behavior Checklist (CBCL). SM initial symptom severity and family therapy compliance, but not duration of SM, contributed to treatment outcomes.

  5. A controlled single-case treatment of severe long-term selective mutism in a child with mental retardation. (United States)

    Facon, Bruno; Sahiri, Safia; Rivière, Vinca


    The aim of the present study was to demonstrate the efficacy of combining two operant learning procedures--shaping and fading--for treating selective mutism. The participant was a 12-year-old boy with mental retardation presenting a severe long-term selective mutism. The treatment was aimed at increasing the loudness of his vocalizations in an increasingly social milieu. The treatment was conducted over the course of about 20 weeks, with four 15-minute sessions per week. A gradual increase in speech loudness was observed. Data indicated a close correspondence between the changes in speech loudness and the criteria for reinforcement successively applied by the therapist, thereby confirming the causal link between the child's progress and the changes in reinforcement contingencies. In addition, good generalization was noted during the stimulus fading phase. Six-month follow up showed that loudness of verbalizations was still satisfactory in the classroom despite a change of school and peer group. The impressive improvement of the child's verbal behavior shows that the implementation of a treatment package including both shaping and stimulus fading is a worthwhile therapeutic option, even in the case of severe long-term selective mutism associated with mental retardation.

  6. Absence of Nails, Deaf-mutism, Seizures, and Intellectual Disability: A Case Report (United States)

    Sagayaraj, Benjamin; Kumar, Radha


    Seizures coexists in children with intellectual disability and are often attributed to neural dysfunction associated with it. Often a careful clinical examination will unravel many diagnostic pointers as in this 8-year-old child with global development delay, deaf-mutism and moderate intellectual disability (mental retardation) who presented with seizures in the emergency department. General examination revealed dysmorphic features like anonychia, low set ears, long philtrum, large lower lips and abnormal dermatoglyphics with features of osteodystrophy on radiology. She was diagnosed as a case of DOORS syndrome, an extremely rare genetic condition affecting the TCA cycle, with just over 40 cases reported, worldwide till date, since its first description in 1961. Her genetic analysis did not reveal the common TBC1D24 mutation in 16p13.3 resulting often from substitutions affecting the arginine at position 242, in spite of all classical clinical features associated with it, suggesting genetic heterogeneity in DOORS syndrome. Though four year follow-up revealed changes in seizure pattern, there was no optic atrophy, change in IQ or peripheral nerve problem. This probably suggests that children with typical clinical features and TBC1D24 mutations may have more progressive deterioration than those without it and newer molecular techniques may identify unexplained phenotypic expressions. PMID:26023614

  7. Behavioral and emotional adjustment, family functioning, academic performance, and social relationships in children with selective mutism. (United States)

    Cunningham, Charles E; McHolm, Angela; Boyle, Michael H; Patel, Sejal


    This study addressed four questions which parents of children with selective mutism (SM) frequently ask: (1) Is SM associated with anxiety or oppositional behavior? (2) Is SM associated with parenting and family dysfunction? (3) Will my child fail at school? and (4) Will my child make friends or be teased and bullied? In comparison to a sample of 52 community controls, 52 children with SM were more anxious, obsessive, and prone to somatic complaints. In contrast, children with SM were less oppositional and evidenced fewer attentional difficulties at school. We found no group differences in family structure, economic resources, family functioning, maternal mood difficulties, recreational activities, or social networks. While parents reported no differences in parenting strategies, children with SM were described as less cooperative in disciplinary situations. The academic (e.g., reading and math) and classroom cooperative skills of children with SM did not differ from controls. Parents and teachers reported that children with SM had significant deficits in social skills. Though teachers and parents rated children with SM as less socially assertive, neither teachers nor parents reported that children with SM were victimized more frequently by peers.

  8. 'More than 100 years of silence', elective mutism: a review of the literature. (United States)

    Sharkey, Louise; McNicholas, Fiona


    Elective mutism is a rare disorder of communication, where the child speaks fluently in familiar situations, such as home, despite lack of speech in less familiar settings, for example school. A variety of temperamental and behaviour characteristics, co-morbid psychiatric conditions, neurodevelopmental delay and family factors have been associated with the disorder. EM children are described as excessively shy, withdrawn, 'slow to warm up', inhibited, often avoid eye contact, fear social embarrassment and experience significant separation anxiety, on separation from their attachment figures. Their behaviour is often perceived by others as controlling and oppositional. Onset of EM is typically in early childhood years. A number of constitutional and environmental factors have been considered in its onset, progression and response to intervention. Treatment is generally considered to be multimodal, and occurs in a variety of settings, including home and school. Longterm studies suggest that communication difficulties may extend into adulthood. In addition, outcome studies showing a high rate of phobic disorders suggest that EM may be a developmental precursor of adult social phobia. This article reviews the literature on EM, its presentation, aetiology, epidemiology and the various evidence based biopsychosocial treatments.

  9. Mutismo cerebelar transitório: relato de dois casos Transitory cerebellar mutism: report of two cases




    Relatamos dois casos de mutismo observados após ressecção de tumores do cerebelo em duas crianças do sexo feminino, tratando-se, no primeiro caso de meduloblastoma, e no segundo, de astrocitoma juvenil. Em ambas havia lesão pré-operatória de nervos bulbares. A fisiopatogenia do mutismo envolve fatores anatômicos, vasculares e emocionais. As características essenciais do mutismo cerebelar são discutidas com base em revisão da literatura.We present two cases of mutism observed after resection o...

  10. Mutismo cerebelar transitório: relato de dois casos Transitory cerebellar mutism: report of two cases

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    Full Text Available Relatamos dois casos de mutismo observados após ressecção de tumores do cerebelo em duas crianças do sexo feminino, tratando-se, no primeiro caso de meduloblastoma, e no segundo, de astrocitoma juvenil. Em ambas havia lesão pré-operatória de nervos bulbares. A fisiopatogenia do mutismo envolve fatores anatômicos, vasculares e emocionais. As características essenciais do mutismo cerebelar são discutidas com base em revisão da literatura.We present two cases of mutism observed after resection of tumors of the cerebellum, in two children of the feminine sex, being in the first case of medulloblastoma and in the second of juvenile astrocytoma. In both patients there was pre-operative lesion of low cranial nerves. The pathophysiology of the mutism involves anatomical, vascular and emotional factors, being its essential characteristics discussed with base in revision of the literature.

  11. Children Who are Anxious in Silence: A Review on Selective Mutism, the New Anxiety Disorder in DSM-5. (United States)

    Muris, Peter; Ollendick, Thomas H


    Selective mutism (SM) is a relatively rare childhood disorder characterized by a consistent failure to speak in specific settings (e.g., school, social situations) despite speaking normally in other settings (e.g., at home). The latest edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-5) lists SM among the anxiety disorders. This makes sense as the current review of the literature confirms that anxiety is a prominent symptom in many children suffering from this condition. Further, research on the etiology and treatment of SM also corroborates the conceptualization of SM as an anxiety disorder. At the same time, critical points can be raised regarding the classification of SM as an anxiety disorder. We explore a number of such issues in this review. Recommendations for dealing with this diagnostic conundrum are made for psychologists, psychiatrists, and other mental health workers who face children with SM in clinical practice, and directions for future research are highlighted.

  12. Mutism and auditory agnosia due to bilateral insular damage--role of the insula in human communication. (United States)

    Habib, M; Daquin, G; Milandre, L; Royere, M L; Rey, M; Lanteri, A; Salamon, G; Khalil, R


    We report a case of transient mutism and persistent auditory agnosia due to two successive ischemic infarcts mainly involving the insular cortex on both hemispheres. During the 'mutic' period, which lasted about 1 month, the patient did not respond to any auditory stimuli and made no effort to communicate. On follow-up examinations, language competences had re-appeared almost intact, but a massive auditory agnosia for non-verbal sounds was observed. From close inspection of lesion site, as determined with brain resonance imaging, and from a study of auditory evoked potentials, it is concluded that bilateral insular damage was crucial to both expressive and receptive components of the syndrome. The role of the insula in verbal and non-verbal communication is discussed in the light of anatomical descriptions of the pattern of connectivity of the insular cortex.

  13. [Cerebellar mutism syndromes with subsequent dysarthria: a study of three children and a review of the literature]. (United States)

    Paquier, P; van Mourik, M; van Dongen, H; Catsman-Berrevoets, C; Brison, A


    Cerebellar mutism and subsequent dysarthria (MSD) is a possible complication of posterior fossa surgery. It is usually seen in children after resection of a cerebellar mass lesion. Most patients become mute after a period of (near)normal postoperative speech, and are dysarthric once speech resumes. The pathophysiological mechanisms underlying MSD are most probably multifactorial, combining neuroanatomical, neurophysiological, neuropsychological, and psychological factors. The aim of the present article is to better define the MSD syndrome. The cerebellum is not only involved in motor control. It is also part of a distributed neural circuitry which underlies higher cognitive functions such as, for instance, those associated with the programming of kinetic parameters before motor initiation of a movement. We hypothesize that it could also be involved in the mental initiation which precedes the programming of any intentional bucco-phonatory movements to be performed in order to express oneself.

  14. Selective mutism due to a dog bite trauma in a 4-year-old girl: a case report

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    Anyfantakis Dimitrios


    Full Text Available Abstract Introduction A child experiencing an event of threatening or catastrophic nature may experience considerable post-traumatic psychological distress. Dog bites present an important public health problem and are a frequent cause of physical trauma in children. Physicians who manage paediatric trauma may not be vigilant of the high risk of psychological stress in children exposed to a physical injury. Case presentation A 4-year-old white girl of Greek origin, with a dog-bite related trauma was admitted to the University Hospital of Crete, Greece, for surgical repair and intravenous antibiotic therapy due to extensive lesions. Exposure to the traumatic event triggered the onset of an unusual psychological response, selective mutism and acute post-traumatic stress disorder. Conclusion There is limited literature discussing the psychological effect of dog bites in children. Parents and physicians involved in pediatric physical trauma need to be more familiar with post-traumatic behavioral reactions. Awareness of the potential development of such reactions may result in early detection and effective management of children at risk.

  15. Selective mutism: a home-and kindergarten-based intervention for children 3-5 years: a pilot study. (United States)

    Oerbeck, Beate; Johansen, Jorunn; Lundahl, Kathe; Kristensen, Hanne


    The aim was to examine the outcome of a multimodal treatment for selective mutism (SM). Seven children, aged three-five years, who were referred for SM were included. The treatment started at home and was continued at kindergarten for a maximum of six months, with predefined treatment goals in terms of speaking levels, from I ("Speaks to the therapist in a separate room with a parent present") through to VI ("Speaks in all kindergarten settings without the therapist present"). The outcome measures were the teacher-reported School Speech Questionnaire (SSQ) and the treatment goal obtained (I-VI) six months after the onset of treatment, and the SSQ and Clinical Global Impression Scale (CGI) at one-year follow-up. Six children spoke in all kindergarten settings (VI) after a mean of 14 weeks treatment. One child, with more extensive neuro-developmental delay, spoke in some settings only (V). The mean SSQ score was 0.59 (SD = 0.51) at baseline compared with 2.68 (SD = 0.35) at the six-month evaluation and 2.26 (SD = 0.93) at one-year follow-up. The mean CGI score at baseline was 4.43 (SD = 0.79) compared with 1.14 (SD = 0.38) at follow-up. Home- and kindergarten-based treatment appears to be promising.

  16. Eficiência da correção endoventricular com patch em pacientes com grande área acinética pós-infarto do miocárdio e severa disfunção ventricular Efficacy of endoventricular patch plasty in large postinfarction akinetic scar and severe left ventricular dysfunction

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    Gustavo Calado de Aguiar RIBEIRO


    Full Text Available OBJETIVO: Verificar a eficiência da técnica de plastia endoventricular com patch circular nos casos com grande área acinética causada por infarto agudo do miocárdio prévio no ventrículo esquerdo e com severa depressão da função ventricular. CASUÍSTICA E MÉTODOS: Este estudo evoluiu resultados hemodinâmicos da plastia endoventricular com patch circular em pacientes com grande área acinética (9 casos e grande área discinética (11 casos e ambos apresentando severa depressão ventricular (fração de ejeção PURPOSE: Efficacy of endoventricular patch plasty in large postinfarction akinetic scar and severe left ventricular dysfunction. METHODS: This study evaluated clinical and hemodynamic results of endoventricular circular patch plasty in patients with either large akinetic scar (n=9 or large dyskinetic scar (n=11 and depressed left ventricular function (ejection fraction < 35%. The difference between akinetic and dyskinetic left ventricular aneurysms was diagnosed by gated radionuclide ventriculography. Groups were comparable for symptons and by echocardiography and by hemodynamic before and after the surgery. RESULTS: Results showed an early improvement in New York Heart Association functional class. Statistically (the Student t test an improvement occurred in left ventricle ejection fraction (from 25 ± 0.8% to 39 ± 1.6% in akinetic patients and from 27 ± 0.7% to 41 ± 1.6% in dyskinetic patients, decreased of capillary wedge pressure (20 ± 1.2 mmHg to 12 ± 1.2 mmHg in akinetics and 17 ± 0,5 mmHg to 11 ± 0.9 mmHg in dyskinetics patients, decrease end-diastolic volume index (226 ± 11 ml to 115 ± 7,8 ml in akinetics and 209 ± 11ml to 96 ± 5ml in dyskinetics and end-systolic volume index (176 ± 9.2 ml to 77 ± 6.2 ml in akinetics and 160 ± 10ml to 66 ± 2ml in dyskinetics patients. Overall operative mortality was 10% (2 cases in akinetic group = 22.2%. CONCLUSION: We find that endoventricular circular patch plasty

  17. Children of Few Words: Relations Among Selective Mutism, Behavioral Inhibition, and (Social) Anxiety Symptoms in 3- to 6-Year-Olds


    Muris, Peter; Hendriks, Eline; Bot, Suili


    Children with selective mutism (SM) fail to speak in specific public situations (e.g., school), despite speaking normally in other situations (e.g., at home). The current study explored the phenomenon of SM in a sample of 57 non-clinical children aged 3–6 years. Children performed two speech tasks to assess their absolute amount of spoken words, while their parents completed questionnaires for measuring children’s levels of SM, social anxiety and non-social anxiety symptoms as well as the tem...

  18. A girl with tuberous sclerosis complex presenting with severe epilepsy and electrical status epilepticus during sleep, and with high-functioning autism and mutism. (United States)

    Pacheva, Iliyana; Panov, Georgi; Gillberg, Christopher; Neville, Brian


    Most patients with tuberous sclerosis complex (TSC) suffer from epilepsy, and many have cognitive and behavioral problems like severe intellectual disability, autism, and hyperactivity. Only rare patients with TSC and autism have a normal intelligence quotient. We report a 13-year-old girl with definite TSC who had early-onset severe epilepsy, autistic behavior, and moderate developmental delay. By school age, however, she had normal intelligence; her intelligence quotient was at least 70 based on a Stanford-Binet test that she refused to complete. She showed good reading, writing, and language comprehension skills, and the special abilities of hyperlexia, hypermnesia, and hypercalculia. However, she did not speak. Criteria of the Diagnostic and Statistical Manual of Mental Disorders, 4th edition, and her Childhood Autism Rating Scale score of 36 indicated mild to moderate autism. She had severe electroencephalographic abnormalities: hypsarrhythmia, multifocal or generalized epileptiform discharges, and electrical status epilepticus during sleep, with a continuous left temporal focus. Magnetic resonance imaging showed many cortical tubers in all brain lobes, and subependymal nodules. We discuss possible explanations for her lack of speech. Considered as speech apraxia, her mutism could be either a symptom of her TSC or a component of her autism. Another possibility is that long-lasting electrical status epilepticus during sleep led to her autistic behavior and language arrest. Still another possibility is that a disinhibited mammalian target of rapamycin (mTOR) pathway was at the root of all of her neuropsychiatric symptoms.

  19. Children of Few Words: Relations Among Selective Mutism, Behavioral Inhibition, and (Social) Anxiety Symptoms in 3- to 6-Year-Olds. (United States)

    Muris, Peter; Hendriks, Eline; Bot, Suili


    Children with selective mutism (SM) fail to speak in specific public situations (e.g., school), despite speaking normally in other situations (e.g., at home). The current study explored the phenomenon of SM in a sample of 57 non-clinical children aged 3-6 years. Children performed two speech tasks to assess their absolute amount of spoken words, while their parents completed questionnaires for measuring children's levels of SM, social anxiety and non-social anxiety symptoms as well as the temperament characteristic of behavioral inhibition. The results indicated that high levels of parent-reported SM were primarily associated with high levels of social anxiety symptoms. The number of spoken words was negatively related to behavioral inhibition: children with a more inhibited temperament used fewer words during the speech tasks. Future research is necessary to test whether the temperament characteristic of behavioral inhibition prompts children to speak less in novel social situations, and whether it is mainly social anxiety that turns this taciturnity into the psychopathology of SM.

  20. Mutilating keratoderma with deaf-mutism

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    Rastogi S


    Full Text Available A 30 year old woman presented with typical lesions of mutilating keratoderma. The patient was deaf and dumb. Hyperkeratosis of palms and soles was present since infancy. Constriction of digits started by the age of 5 years. The clinical diagnosis was supported by histopathological examination.

  1. Behavior therapy in a family context: treating elective mutism. (United States)

    Rosenberg, J B; Lindblad, M B


    This paper discusses the necessity of using both behavioral and family approaches in combination, while working with electively mute children. The symptom and its significance within the family system is presented along with a rationale for avoiding the pitfalls of individual approaches with such children. A case history outlining specific behavioral techniques is described in detail with an exploration of the use of reinforcement theory, counter-conditioning, and successive approximations in bringing about change in electively mute children. The need for bringing about changes within the family system so as to maintain the changes that have occurred through use of the behavior techniques is discussed and presented as crucial to the treatment process. The paper takes the position that either approach, by itself, will not be effective in helping electively mute children but that the treatment of choice is a combination of therapeutic techniques.

  2. Atypical Parkinsonism Revealing a Late Onset, Rigid and Akinetic Form of Huntington's Disease

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    A. Ciammola


    Full Text Available Huntington's disease (HD is a rare hereditary neurodegenerative disorder characterized in over 90 percent of cases by chorea as the presenting motor symptom. We report a 54-year-old male who presented with Parkinsonism as the initial symptom of the disease. Genetic analysis revealed expansion of 40 CAG repeats, and brain MRI showed both severe caudate nuclei and cortical atrophy. Single-photon emission computed tomography (SPECT imaging of the dopamine transporter showed nigrostriatal pathway degeneration. Here, we also describe his 2 years of clinical followup after ensuing dopaminergic stimulation.

  3. Bruxism Associated with Anoxic Encephalopathy: Successful Treatment with Baclofen (United States)

    Janati, A. Bruce; ALGhasab, Naif Saad; ALGhassab, Fahad Saad


    Introduction. Bruxism is a movement disorder characterized by grinding and clenching of the teeth. Etiology of bruxism can be divided into three groups: psychosocial factors, peripheral factors, and pathophysiological factors. Methods. The clinical investigation was conducted at King Khaled Hospital in Hail, Saudi Arabia, in 2012. Results. A 16-year-old Saudi female was brought to the hospital in a comatose state and with generalized convulsive seizures secondary to acute anoxic encephalopathy. In the third week of hospitalization, while still in a state of akinetic mutism, she developed incessant bruxism which responded favorably to a GABA receptor agonist (baclofen). Conclusion. Our data support the hypothesis that bruxism emanates from imbalance or dysregulation of the neurotransmitter system. Larger scale studies will be needed to confirm this hypothesis. PMID:24455317

  4. Bruxism Associated with Anoxic Encephalopathy: Successful Treatment with Baclofen

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    A. Bruce Janati


    Full Text Available Introduction. Bruxism is a movement disorder characterized by grinding and clenching of the teeth. Etiology of bruxism can be divided into three groups: psychosocial factors, peripheral factors, and pathophysiological factors. Methods. The clinical investigation was conducted at King Khaled Hospital in Hail, Saudi Arabia, in 2012. Results. A 16-year-old Saudi female was brought to the hospital in a comatose state and with generalized convulsive seizures secondary to acute anoxic encephalopathy. In the third week of hospitalization, while still in a state of akinetic mutism, she developed incessant bruxism which responded favorably to a GABA receptor agonist (baclofen. Conclusion. Our data support the hypothesis that bruxism emanates from imbalance or dysregulation of the neurotransmitter system. Larger scale studies will be needed to confirm this hypothesis.

  5. [Inpatient treatment of a child with elective mutism--a case report]. (United States)

    Wernitznig, H


    The paper presents the case of a 10-year-old, electively mute boy, who had stopped talking with all persons outside his immediate family about four years ago. Therapeutic interventions had so far brought no change in his speech behavior. Within a short time (2 months) of residential treatment in a child care unit, his mutistic disorder and social behavior could be normalized and the child even managed to speak in this former school class where nobody had ever heard his voice. The multitude of possible reasons for the mutistic behavior and reinforcing environmental facts are reviewed, the symptom's meaning for the family system considered. The plan of treatment (mostly a combination of behavioral and systemic family therapy) is presented.

  6. Tumour type and size are high risk factors for the syndrome of "cerebellar" mutism and subsequent dysarthria

    NARCIS (Netherlands)

    C.E. Catsman-Berrevoets (Coriene); H.R. van Dongen (Hugo); D. Paz y Geuze; P.F. Paquier; M.H. Lequin (Maarten); P.G.H. Mulder (Paul)


    textabstractOBJECTIVE: "Cerebellar mutis" and subsequent dysarthria (MSD) is a documented complication of posterior fossa surgery in children. In this prospective study the following risk factors for MSD were assessed: type, size and site of the tumour; hydrocephalus at

  7. Tumour type and size are high risk factors for the syndrome of "cerebellar" mutism and subsequent dysarthria



    textabstractOBJECTIVE: "Cerebellar mutis" and subsequent dysarthria (MSD) is a documented complication of posterior fossa surgery in children. In this prospective study the following risk factors for MSD were assessed: type, size and site of the tumour; hydrocephalus at presentation and after surgery, cerebellar incision site, postoperative infection, and cerebellar swelling. METHODS: In a consecutive series of 42 children with a cerebellar tumour, speech and neuroradiological studies (CT and...

  8. Can prion disease suspicion be supported earlier? Clinical, radiological and laboratory findings in a series of cases. (United States)

    González-Duarte, Alejandra; Medina, Zaira; Balaguer, Rainier Rodriguez; Calleja, Jesus Higuera


    The subacute spongiform encephalopathies are prion diseases characterized by acute and rapid neurodegeneration that lead to the death of the patient within months to a few years. The epidemiology of CJD is complicated and the frequency in Mexico is unknown. We aim to describe the cases of prion disease in Mexico. Consecutive patients who met the diagnostic criteria by the WHO were enrolled. We describe 26 patients with clinical manifestations, imaging and laboratory studies compatible with prion disease. The mean age at onset was 52 years old. The main clinical manifestations were cognitive alterations (69%) followed by extrapyramidal movements (50%), abnormal cerebellar function (46%), behavioral alterations (46%), myoclonus (46%), and mood depression (23%), among other features. Half of the patients progressed rapidly to a state of akinetic mutism (53%). Only 2 (7.6%) patients had a family history of a similar disease. Time interval between onset and diagnosis varied between 71 days to 24 months, with a median of 6 months. The classical bilateral basal ganglia hyperintensities were present in the very early stage of the disease. Protein 14-3-3 immuneassay in the CSF was positive in all measured cases. Bilateral basal ganglia hyperintensities was the most important early finding, while protein 14-3-3 was a late finding and the results were usually obtained after the patient was discharged. Around 1.5 cases of CJD cases per year are reported in our country. When suspected, MRI can support the diagnosis earlier than other studies.

  9. Stereotypic Movements in Case of Sporadic Creutzfeldt-Jakob Disease: Possible Role of Anti-NMDA Receptor Antibodies

    Directory of Open Access Journals (Sweden)

    Michelle Molina


    Full Text Available Sporadic Creutzfeldt-Jakob disease (sCJD and anti-NMDA receptor antibody encephalitis (NMDAE can both produce a rapidly progressive dementia with resulting state of catatonia or akinetic mutism. Both are associated with movement disorders. In published case series, myoclonus appears to be the most frequent movement disorder in sCJD, while stereotypic, synchronized, one-cycle-per-second movements such as arm or leg elevation, jaw opening, grimacing, head turning, and eye deviation are seen in NMDAE. We report a case of a 59-year-old woman with rapidly worsening cognitive disturbance leading to a nearly catatonic state interrupted by stereotypic movements. sCJD was diagnosed via periodic sharp wave complexes on EEG as well as cerebrospinal fluid (CSF 14-3-3 and tau protein elevation. Characteristic movement disorder of NMDAE was present in absence of ovarian mass or CSF pleiocytosis. Given prior case reports of presence of anti-NMDA receptor antibodies in sCJD, we propose that the movement disorder in this case was caused by anti-NMDA receptor antibodies whose formation was secondary to neuronal damage from prion disease. It is important to consider sCJD even in cases that have some clinical features suggestive of NMDAE.

  10. Panencephalopathic Creutzfeldt-Jakob disease with distinct pattern of prion protein deposition in a patient with D178N mutation and homozygosity for valine at codon 129 of the prion protein Gene. (United States)

    Marcon, Gabriella; Indaco, Antonio; Di Fede, Giuseppe; Suardi, Silvia; Finato, Nicoletta; Moretti, Valentino; Micoli, Sandro; Fociani, Paolo; Zerbi, Pietro; Pincherle, Alessandro; Redaelli, Veronica; Tagliavini, Fabrizio; Giaccone, Giorgio


    Prion diseases include sporadic, acquired and genetic forms linked to mutations of the prion protein (PrP) gene (PRNP). In subjects carrying the D178N PRNP mutation, distinct phenotypes can be observed, depending on the methionine/valine codon 129 polymorphism. We present here a 53-year-old woman with D178N mutation in the PRNP gene and homozygosity for valine at codon 129. The disease started at age 47 with memory deficits, progressive cognitive impairment and ataxia. The clinical picture slowly worsened to a state of akinetic mutism in about 2 years and the disease course was 6 years. The neuropathologic examination demonstrated severe diffuse cerebral atrophy with neuronal loss, spongiosis and marked myelin loss and tissue rarefaction in the hemispheric white matter, configuring panencephalopathic Creutzfeldt-Jakob disease. PrP deposition was present in the cerebral cortex, basal ganglia and cerebellum with diffuse synaptic-type pattern of immunoreactivity and clusters of countless, small PrP deposits, particularly evident in the lower cortical layers, in the striatum and in the molecular layer of the cerebellum. Western blot analysis showed the presence of type 1 PrP(Sc) (Parchi classification). These findings underline the clear-cut distinction between the neuropathological features of Creutzfeldt-Jakob disease associated with D178N PRNP mutation and those of fatal familial insomnia.

  11. A case of Creutzfeldt-Jakob disease: diagnostic dilemmas of a rapidly fatal disease

    Directory of Open Access Journals (Sweden)

    Mirza M. Baig


    Full Text Available Creutzfeldt-Jakob disease (CJD is a rapidly progressive and ultimately fatal disorder of the central nervous system. It occurs worldwide with an incidence of 0.5-1 new case per million population per year. No specific treatment is available and management is limited to supportive care. Autopsy or biopsy provides a definitive diagnosis. Because of the transmissible nature of the disease and hesitancy of patients/family members to give consent for biopsy, numerous challenges in confirming the clinical diagnosis are faced by healthcare professionals. We report a case of 66-year-old male who was hospitalized due to hip fracture following a fall. Acute mental status changes followed the surgical fixation of hip fracture which triggered neurologic work up. This finally revealed suspicion and confirmation of CJD. Patient had progressive cognitive decline with akinetic mutism during further hospital stay and was later discharged home with hospice. Shorter thereafter he died at home. This case demonstrates the importance of keeping an open mind towards possibility of CJD when faced with esoteric neurologic presentations. Also this case provides insight into challenges in quarantine and sterilization of surgical instruments when these patients go through major surgeries.

  12. Can prion disease suspicion be supported earlier? (United States)

    Medina, Zaira; Balaguer, Rainier Rodriguez; Calleja, Jesus Higuera


    The subacute spongiform encephalopathies are prion diseases characterized by acute and rapid neurodegeneration that lead to the death of the patient within months to a few years. The epidemiology of CJD is complicated and the frequency in Mexico is unknown. We aim to describe the cases of prion disease in Mexico. Consecutive patients who met the diagnostic criteria by the WHO were enrolled. We describe 26 patients with clinical manifestations, imaging and laboratory studies compatible with prion disease. The mean age at onset was 52 years old. The main clinical manifestations were cognitive alterations (69%) followed by extrapyramidal movements (50%), abnormal cerebellar function (46%), behavioral alterations (46%), myoclonus (46%) and mood depression (23%), among other features. Half of the patients progressed rapidly to a state of akinetic mutism (53%). Only 2 (7.6%) patients had a family history of a similar disease. Time interval between onset and diagnosis varied between 71 days to 24 months, with a median of 6 months. The classical bilateral basal ganglia hyperintensities were present in the very early stage of the disease. Protein 14-3-3 immuneassay in the CSF was positive in all measured cases. Bilateral basal ganglia hyperintensities was the most important early finding, while protein 14-3-3 was a late finding and the results were usually obtained after the patient was discharged. Around 1.5 cases of CJD cases per year are reported in our country. When suspected, MRI can support the diagnosis earlier than other studies. PMID:21869605

  13. 小脑性缄默和继发构音障碍综合征%Syndrome of cerebellar mutism and subsequent dysarthria

    Institute of Scientific and Technical Information of China (English)

    吐尔逊·肉苏力; 朱国华; 杜郭佳; 吴昊; 汪永新


    @@ 小脑性缄默是指继发于小脑肿瘤术后的暂时性不能讲话,但发音器官正常,恢复后常伴有构音障碍.本文报道2004年1月至2009年6月收治的8例小脑性缄默症如下: 一、对象与方法 1. 一般资料:本组8例患者,男3例,女5例;年龄3~14岁.术前主要症状为头痛、恶心、呕吐;共济失调4例.水平眼震3例,所有患者均有眼底视乳头水肿.

  14. Tc-99m-bicisate (ECD)-brain-SPECT in rapidly progressive dementia; Hirn-SPECT mit Tc-99m-Bicisat (ECD) bei rasch progredientem dementiellen Syndrom

    Energy Technology Data Exchange (ETDEWEB)

    Marienhagen, J.; Eilles, C. [Regensburg Univ. (Germany). Abt. fuer Nuklearmedizin; Weingaertner, U.; Blaha, L. [Bezirkskrankenhaus Mainkofen (Germany). Psychiatrische Klinik; Zerr, I.; Poser, S. [Goettingen Univ. (Germany). Klinik und Poliklinik fuer Neurologie


    We present a 61-year-old male patient with progressive dementia. A brain SPECT with Tc-99m-bicisate was performed for confirmation of clinically suspected Alzheimer-dementia. At the time of the SPECT-investigation marked apraxia and aphasia besides severe dementia were present. Electrophysiological as well as anatomical neuroimaging findings showed non-diagnostic alterations. SPECT revealed distinct perfusion defects, which made Alzheimer Dementia unlikely. The further course of the patient was determined by rapidly progressive deterioration with development of akinetic mutism. Thereafter, increased levels of neuron-specific enolase as well as 14-3-3 proteins were found in the cerebro-spinal fluid (CSF). The patient finally died with signs of cerebral decortication. Due to the clinical course and the CSF-findings the patient's final diagnosis was Creutzfeld-Jakob-disease, nevertheless no autopsy was performed. The presented case report underscores the clinical utility of perfusion brain SPECT in the differential diagnosis of dementias. (orig.) [German] Wir berichten ueber einen 61jaehrigen Patienten mit progredientem dementiellen Syndrom, der unter der Verdachtsdiagnose einer Demenz vom Alzheimer-Typ (DAT) zur Hirn-SPECT-Untersuchung mit TC-99m-Bicisat (ECD) vorgestellt wurde. Zum Untersuchungszeitpunkt bestanden neben dem Vollbild einer Demenz eine ausgepraegte Apraxie und Aphasie bei unspezifischen Veraenderungen im EEG sowie der neuroradiologischen Bildgebung. In der Hirn-SPECT-Untersuchung fanden sich fuer eine DAT untypische ausgedehnte, vorwiegend rechtshemisphaerische Perfusionsstoerungen. Im weiteren Verlauf rasche Progredienz des Krankheitsbildes mit Entwicklung eines akinetischen Mutismus sowie Nachweis erhoehter Werte der neuronspezifischen Enolase und des 14-3-3-Proteins im Liquor. Der Patient verstarb schliesslich unter dem Bild einer Decortication. Aufgrund des klinischen Verlaufs sowie der Liquorbefunde wurde, da eine autoptische Befundsicherung

  15. Novel mutation of the PRNP gene of a clinical CJD case

    Directory of Open Access Journals (Sweden)

    Collinge John


    Full Text Available Abstract Background Transmissible spongiform encephalopathies (TSEs, a group of neurodegenerative diseases, are thought to be caused by an abnormal isoform of a naturally occurring protein known as cellular prion protein, PrPC. The abnormal form of prion protein, PrPSc accumulates in the brain of affected individuals. Both isoforms are encoded by the same prion protein gene (PRNP, and the structural changes occur post-translationally. Certain mutations in the PRNP gene result in genetic TSEs or increased susceptibility to TSEs. Case presentation A 70 year old woman was admitted to the hospital with severe confusion and inability to walk. Relatives recognized memory loss, gait and behavioral disturbances over a six month period prior to hospitalization. Neurological examination revealed Creutzfeldt-Jakob disease (CJD related symptoms such as incontinence, Babinski sign and myoclonus. EEG showed periodic sharp waves typical of sporadic CJD and cerebrospinal fluid analysis (CSF was positive for the presence of the 14-3-3-protein. As the disease progressed the patient developed akinetic mutism and died in the tenth month after onset of the disease symptoms. Unfortunately, no autopsy material was available. PRNP sequencing showed the occurrence of a point mutation on one allele at codon 193, which is altered from ACC, coding for a threonine, to ATC, encoding an isoleucine (T193I. Conclusion Here we report a novel mutation of the PRNP gene found in an elderly female patient resulting in heterozygosity for isoleucine and threonine at codon 193, in which normally homozygosity for threonine is expected (T193. The patient presented typical clinical symptoms of CJD. EEG findings and the presence of the 14-3-3 protein in the CSF, contributed to CJD diagnosis, allowing the classification of this case as a probable CJD according to the World Health Organization (WHO accepted criteria.

  16. An autopsy-verified case of FTLD-TDP type A with upper motor neuron-predominant motor neuron disease mimicking MM2-thalamic-type sporadic Creutzfeldt-Jakob disease. (United States)

    Hayashi, Yuichi; Iwasaki, Yasushi; Takekoshi, Akira; Yoshikura, Nobuaki; Asano, Takahiko; Mimuro, Maya; Kimura, Akio; Satoh, Katsuya; Kitamoto, Tetsuyuki; Yoshida, Mari; Inuzuka, Takashi


    Here we report an autopsy-verified case of frontotemporal lobar degeneration (FTLD)-transactivation responsive region (TAR) DNA binding protein (TDP) type A with upper motor neuron-predominant motor neuron disease mimicking MM2-thalamic-type sporadic Creutzfeldt-Jakob disease (sCJD). A 69-year-old woman presented with an 11-month history of progressive dementia, irritability, insomnia, and gait disturbance without a family history of dementia or prion disease. Neurological examination revealed severe dementia, frontal signs, and exaggerated bilateral tendon reflexes. Periodic sharp-wave complexes were not observed on the electroencephalogram. Brain diffusion MRI did not reveal abnormal changes. An easy Z score (eZIS) analysis for (99m)Tc-ECD-single photon emission computed tomography ((99m)Tc-ECD-SPECT) revealed a bilateral decrease in thalamic regional cerebral blood flow (rCBF). PRNP gene analysis demonstrated methionine homozygosity at codon 129 without mutation. Cerebrospinal fluid (CSF) analysis showed normal levels of both 14-3-3 and total tau proteins. Conversely, prion protein was slowly amplified in the CSF by a real-time quaking-induced conversion assay. Her symptoms deteriorated to a state of akinetic mutism, and she died of sudden cardiac arrest, one year after symptom onset.  Despite the SPECT results supporting a clinical diagnosis of MM2-thalamic-type sCJD, a postmortem assessment revealed that this was a case of FTLD-TDP type A, and excluded prion disease. Thus, this case indicates that whereas a bilateral decreasing thalamic rCBF detected by (99m)Tc-ECD-SPECT can be useful for diagnosing MM2-thalamic-type sCJD, it is not sufficiently specific. Postmortem diagnosis remains the gold standard for the diagnosis of this condition.

  17. Increasing Verbal Behavior of a Student Who Is Selectively Mute (United States)

    Beare, Paul; Torgerson, Colleen; Creviston, Cindy


    "Selective mutism" is the term used to describe a disorder in which a person speaks only in restricted stimulus situations. Examination of single-subject research concerning selective mutism reveals the most popular and successful interventions to instate speech involve a combination of behavior modification procedures. The present research…

  18. [Functional pathophysiology of consciousness]. (United States)

    Jellinger, Kurt A


    from important somatic and sensory pathways and acts as a control system of neuronal activities of the cerebral cortex. The principal function of the ARAS is to focus our alertness on specific stimuli or internal processes, which run via complex neuronal cell groups and numerous neurotransmitters that influence various aspects of consciousness and wakefulness. Stimulation of the ARAS produces an arousal reaction as the electric correlate of consciousness; its destruction causes coma and related states. The highest level are cortical (prefrontal and association) networks for recognition, motor activity, longterm memory and attention, the left hemisphere being considered as the dominant one. Different levels of consciousness are distinguished: 1. hyperalertness, 2. alertness (normal state of wakefulness), 3. somnolence or lethargy, 4. obtundation with tendency to fall asleep, 5. stupor, 6. coma and its subtypes, like akinetic mutism, apallic syndrome or persistent vegative state, locked-in syndrome, delirium, and catatonia. They are caused by damages in various functional levels of the brain, by psychogenic factors or experimentally, and are accompanied by characteristic neurological and psychiatric disorders. The relevant morphological lesions can be detected by electrophysiological and imaging studies. The bases of functional anatomy and pathophysiology of consciousness, its cognitive aspects and its major disorders, their causes and functional substrates with reference to sleep and both spontaneous and iatrogenic disorders of consciousness are critically summarized.

  19. Creutzfeldt-Jakob病43例患者的临床分析%The clinical features of 43 patients with Creutzfeldt-Jakob disease

    Institute of Scientific and Technical Information of China (English)

    刘静; 王玉平; 王红星; 李莉萍; 刘爱华; 叶静; 杨延辉


    Objective To analyze the clinical features of 43 patients with clinically possible or probable Creutzfeldt-Jakob disease (CJD) to provide references for the early clinical evaluation and diagnosis of CJD. Method All patients who were diagnosed with “suspected CJD” and hospitalized in our hospital between January 2013 to January 2016 were collected, and their clinical features and laboratory data were analyzed retrospectively. Results In general, the onset of CJD occurred at about 60 years old, with a mean course of disease of 5.70±5.08 months(median:four months). The first symptoms are changeable, mainly presenting with rapidly progressive dementia. In addition, typical clinical manifestations included 6 types: rapidly progressivedementia, damage in the locomotor system(fibrae pyramidales, extrapyramidal and cerebellar symptoms), myoclonus, akinetic mutism, sleep and vision disorders.Conclusion The early diagnosis of CJD should pay attention to its clinical features, and highly alarming and periodic review should be given to the patient with 2 typical features or above manifestations to avoid misdiagnosis, although 14-3-3 protein, EEG and MRI show atypical findings.%目的:分析43例临床可能或很可能克雅氏病(CJD)患者的临床特征,为CJD早期诊断提供一些参考。方法搜集2013年1月至2016年1月以“可疑CJD”诊断在首都医科大学宣武医院住院的患者,对其临床特点及实验室资料进行分析。结果 CJD通常在60岁左右发病,平均病程5.70±5.08个月;首发症状多变,以迅速进展性痴呆为主。典型临床表现有6种:迅速进展的痴呆、运动系统损害(锥体束、锥体外系及小脑症状)、肌阵挛、无动性缄默、睡眠障碍和视力障碍。结论 CJD的早期诊断应重视其临床特征,当一个患者具有典型特征中的两项或以上表现时,即使14-3-3蛋白、脑电图(EEG)、磁共振(MRI)均不典型,也要高度警惕CJD

  20. Absence of Neurobehavioral Disturbance in a Focal Lesion of the Left Paracentral Lobule

    Directory of Open Access Journals (Sweden)

    T. Imamura


    Full Text Available The case of a right-handed woman with an infarcation confined to the left paracentral lobule and sparing the supplementary motor area (SMA is reported. She presented with a right leg monoplegia and displayed no mutism. The absence of any associated neurobehavioral disturbances (mutism, forced grasping, reduced spontaneous arm activity or aphasia raises the possibility that the left SMA has discrete neurobehavioral functions.

  1. On Cognitive Strategies for Facilitating Acquisition, Retention, and Retrieval in Training and Education (United States)


    mechanisms underlying the strategy of actions (e.g., hypo- thalamus, amygdala, and orbitofrontal cortex) receive, of course, exteroceptive in addition...mutism. The syndrome is char- acterized by absolute mutism and complete immobility except for the eyes, which are usually kept open and moved in...Cognition, 1976, 4, 103-108. Hess, W.R. Das Zwischenhirn. Syndrome , LokalisatIonen, Funktionen. Basel: B. Schwabe, 1949. Hess, E.H. Attitude and


    NARCIS (Netherlands)



    In addition to impaired dopaminergic neurotransmission a dysfunctional noradrenergic system has been demonstrated in Parkinson's disease. L-threo-3,4-dihydroxyphenylserine (DOPS), a synthetic precursor of noradrenaline (NA), appears to be effective in the treatment of some akinetic symptoms in parki

  3. Iodine deficiency, thyroid function and hearing deficit: a review

    NARCIS (Netherlands)

    Boonstra, A.; MacKenzie, I.


    Iodine deficiency affects an estimated 241 million school-aged children in the world. Little is known about iodine deficiency in relation to auditory function, except for the fact that deaf-mutism is one of the features of cretinism. In the present review, we documented the scientific knowledge on t

  4. Transient Stuttering in Catatonic Bipolar Patients

    Directory of Open Access Journals (Sweden)

    Anthony B. Joseph


    Full Text Available Two cases of transient stuttering occurring in association with catatonia and bipolar disorder are described. Affective decompensation has been associated with lateralized cerebral dysfunction, and it is hypothesized that in some bipolar catatonic patients a concomitant disorder of the lateralization of language function may lead to a variety of clinical presentations including aphasia, mutism, and stuttering.

  5. Catatonia in Autism: A Distinct Subtype? (United States)

    Ghaziuddin, M.; Quinlan, P.; Ghaziuddin, N.


    Catatonia is a life-threatening disorder characterized by motor abnormalities, mutism, and disturbances of behaviour, which is increasingly being diagnosed in persons with autism. In this report, we describe the presentation and course of catatonia in an adolescent with autism who responded to electroconvulsive therapy (ECT). The illness started…

  6. Cannabis Induced Periodic Catatonia: A Case Report (United States)

    Bajaj, Vikrant; Pathak, Prashant; Mehrotra, Saurabh; Singh, Vijender; Govil, Sandeep; Khanna, Aman


    Catatonia is a syndrome of specific motor abnormalities closely associated with disorders in mood, affect, thought and cognition. The principal signs of the disorder are mutism, immobility, negativism, posturing, stereotypy and echo phenomena. Catatonia is commonly seen in various psychiatric disorders, neurological disorders and certain medical…

  7. [Autism and catatonia: successful treatment using lorazepam. A case study]. (United States)

    de Winter, C F; van Dijk, F; Verhoeven, W M A; Dhossche, D M; Stolker, J J


    A 24-year-old man who was mentally retarded and had an autistic disorder, developed mutism and motor symptoms. He was diagnosed with catatonia and was treated successfully with lorazepam. Additionally, we review the literature about the diagnosis and treatment of catatonia in patients with autism; in such cases accurate diagnosis is vital but is complicated by overlapping symptoms.



    Santosh Kumar; Sunil Kumar; Anand


    Waardenburg syndrome is a rare syndrome, characterized by lateral displacement of the medial canthi combined with dystopia of the lacrimal punctum and blepharophimosis, prominent broad nasal root, hypertrichosis of the medial part of the eyebrows, white forelock, heterochromia iridis, and deaf mutism. A four months old girl with waardenburg syndrome type II, who had the characterstic features of the syndrome, is reported.

  9. Echolalia, Mitigation and Autism: Indicators from Child Characteristics for the Use of Sign Language and Other Augmentative Language Systems. (United States)

    Bebko, James M.


    Review of literature on indicators of the effectiveness of language intervention programs for autistic children showed that mitigation in echolalia was a critical characteristic, as it implied that the prerequisites for language were accessible through speech. Children whose speech ranged from mutism to unmitigated echolalia had a more negative…

  10. Symbolization Levels in Communicative Behaviors of Children Showing Pervasive Developmental Disorders. (United States)

    Atlas, Jeffrey A.; Lapidus, Leah Blumberg


    A total of 48 children (aged 4-14) with severe pervasive developmental disturbance, exhibiting mutism, echolalia, or nonecholalic speech, were observed in their communicative behaviors across modalities. Levels of symbolization in gesture, play, and drawing were significantly intercorrelated and were most strongly correlated with the criterion…

  11. Insights from the supplementary motor area syndrome in balancing movement initiation and inhibition

    NARCIS (Netherlands)

    Potgieser, A. R. E.; de Jong, BM; Wagemakers, M.; Hoving, E. W.; Groen, R. J. M.


    The supplementary motor area (SMA) syndrome is a characteristic neurosurgical syndrome that can occur after unilateral resection of the SMA. Clinical symptoms may vary from none to a global akinesia, predominantly on the contralateral side, with preserved muscle strength and mutism. A remarkable fea

  12. Role of Cerebellum in Fine Speech Control in Childhood: Persistent Dysarthria after Surgical Treatment for Posterior Fossa Tumour (United States)

    Morgan, A. T.; Liegeois, F.; Liederkerke, C.; Vogel, A. P.; Hayward, R.; Harkness, W.; Chong, K.; Vargha-Khadem, F.


    Dysarthria following surgical resection of childhood posterior fossa tumour (PFT) is most commonly documented in a select group of participants with mutism in the acute recovery phase, thus limiting knowledge of post-operative prognosis for this population of children as a whole. Here we report on the speech characteristics of 13 cases seen…

  13. Huntington Disease: A Case Study of Early Onset Presenting as Depression (United States)

    Duesterhus, Pia; Schimmelmann, Benno Graf; Wittkugel, Oliver; Schulte-Markwort, Michael


    Huntington disease is a dominantly inherited, neurodegenerative disease characterized by choreiform movement disturbances and dementia, usually with adult onset. The rare juvenile-onset Huntington disease differs from the adult phenotype. A case presenting twice, at age 10 with all the signs of a major depression and age 14 with mutism and…

  14. Narrative Counseling for Professional School Counselors (United States)

    Nafziger, Jacinta; DeKruyf, Lorraine


    This article introduces narrative counseling concepts and techniques for professional school counselors. The authors provide a case study of narrative school counseling with an elementary student struggling with selective mutism. Examples also demonstrate how a narrative approach could be used at elementary, middle, and high school levels within…

  15. Electroconvulsive therapy as a treatment for refractory neuropsychiatric lupus with catatonia: three case studies and literature review. (United States)

    Bica, B E R G; Moro, A L D; Hax, V; Nicol, N A; Campos, G S; Rivera, L M S; da Costa, A F C; Xavier, R M; Monticielo, O A


    Neuropsychiatric disorders associated with systemic lupus erythematosus are very common. Treatment generally consists of glucocorticoids and immunosuppressive therapy; however, some cases are unresponsive. Electroconvulsive therapy (ECT) is a recognized treatment modality in psychiatry and is an option for refractory cases of neuropsychiatric lupus. This report describes three cases of neuropsychiatric lupus that improved with ECT after failure of antipsychotics and immunosuppressive therapy. All cases met DSM-5 criteria for catatonia (case 1: agitation, stereotypies, and grimacing; case 2: stupor, mutism, and grimacing; case 3: agitation, mutism, and stereotypies); therefore, ECT was indicated. This case series shows that ECT can be a therapeutic option in patients with neuropsychiatric lupus, especially when associated with catatonia and unresponsive to conventional treatment.

  16. Psychosis secondary to tuberculosis meningitis. (United States)

    Rahim, Mohd Jazman Che; Ghazali, Wan Syamimee Wan


    We report a case of a 19-year-old immunocompetent Malay woman who presented with a worsening psychotic disorder of 1-year duration. She initially presented with social isolation with subsequent mutism and stupor. Physical examination revealed a stuporous, emaciated, dehydrated woman with Glasgow Coma Scale of 11/15 (E4V2M5). She had a blank stare, mutism and akinesia. Motor examination revealed upper motor neuron findings. Neck stiffness was present, however, Kernig's and Brudzinski's signs were negative. There were no other findings on other systems. Brain imaging and EEG were normal. Cerebrospinal fluid investigations revealed positive cerebrospinal fluid Mycobacterium tuberculosis PCR (MTB PCR). The patient was treated with empirical antituberculosis drugs and steroids. On follow-up visit 1 month later, her psychotic symptoms had fully resolved. She was able to ambulate and care for herself; she was unable to recall the symptoms she had experienced before and during admission.

  17. Auditory-perceptual speech analysis in children with cerebellar tumours: a long-term follow-up study. (United States)

    De Smet, Hyo Jung; Catsman-Berrevoets, Coriene; Aarsen, Femke; Verhoeven, Jo; Mariën, Peter; Paquier, Philippe F


    Mutism and Subsequent Dysarthria (MSD) and the Posterior Fossa Syndrome (PFS) have become well-recognized clinical entities which may develop after resection of cerebellar tumours. However, speech characteristics following a period of mutism have not been documented in much detail. This study carried out a perceptual speech analysis in 24 children and adolescents (of whom 12 became mute in the immediate postoperative phase) 1-12.2 years after cerebellar tumour resection. The most prominent speech deficits in this study were distorted vowels, slow rate, voice tremor, and monopitch. Factors influencing long-term speech disturbances are presence or absence of postoperative PFS, the localisation of the surgical lesion and the type of adjuvant treatment. Long-term speech deficits may be present up to 12 years post-surgery. The speech deficits found in children and adolescents with cerebellar lesions following cerebellar tumour surgery do not necessarily resemble adult speech characteristics of ataxic dysarthria.

  18. Characterization of the Pathological and Biochemical Markers that Correlate to the Clinical Features of Autism (United States)


    vary from mutism to verbal fl uency (Rapin, 1996; Stone et al., 1997; Wetherby et al., 1998). Sensorimotor defi cits also show signifi cant...cells and, to a lesser extent, of granule cells, was pres- ent in gracile, tonsil , and inferior semilunar lobules. Changes were not detected in...semantics) or language and communicative defi cits in social context (social pragmatics) (Rapin, 1996; Stone et al., 1997; Wetherby et al., 1998

  19. Organisk kataton tilstand efter apopleksi

    DEFF Research Database (Denmark)

    Jørgensen, Anders; Jørgensen, Martin Balslev


    Catatonia is seen in various psychiatric disorders, but also rarely occurs in medical conditions with organic cerebral affection. We present a case of a previously mentally healthy male, who developed catatonia years after a stroke in the right hemisphere. Catatonic symptoms included stupor, mutism......, stereotyped movements and repetition of meaningless sounds. The condition responded to benzodiazepine and electroconvulsive therapy. Catatonia should be considered as a differential diagnosis when the described symptoms occur in patients with a known organic cerebral disorder. Udgivelsesdato: 2009-Aug...

  20. Catatonia: Our current understanding of its diagnosis, treatment and pathophysiology


    Rasmussen, Sean A; Mazurek, Michael F.; Rosebush, Patricia I.


    Catatonia is a psychomotor syndrome that has been reported to occur in more than 10% of patients with acute psychiatric illnesses. Two subtypes of the syndrome have been identified. Catatonia of the retarded type is characterized by immobility, mutism, staring, rigidity, and a host of other clinical signs. Excited catatonia is a less common presentation in which patients develop prolonged periods of psychomotor agitation. Once thought to be a subtype of schizophrenia, catatonia is now recogni...


    Directory of Open Access Journals (Sweden)

    Santosh Kumar


    Full Text Available Waardenburg syndrome is a rare syndrome, characterized by lateral displacement of the medial canthi combined with dystopia of the lacrimal punctum and blepharophimosis, prominent broad nasal root, hypertrichosis of the medial part of the eyebrows, white forelock, heterochromia iridis, and deaf mutism. A four months old girl with waardenburg syndrome type II, who had the characterstic features of the syndrome, is reported.

  2. Waardenburg's syndrome: case reports in two Nigerians. (United States)

    Amoni, S S; Abdurrahman, M B


    We saw two cases of Waardenburg's syndrome in Nigerian children. A 9-year-old girl had congenital deaf-mutism, high-arched palate, complete heterochromia iridis with pigment mottling in the posterior pole of the eye with the hypochromic iris, dyspigmented frontal scalp hair, a history of vitiligo, but with no dystopia canthorum. An 11-year-old boy had a harelip with cleft palate, heterochromia iridis, blonde fundus on the eye with blue iris, and dystopia canthorum.

  3. Quadrigeminal plate lipoma presenting with Psychosis: A case ‎report with review of literature

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    Sourav Das


    Full Text Available A young patient who presented with headache followed by positive and negative symptoms of psychosis and mutism was sent for the MRI of brain. MRI revealed a lipoma in the quardrigeminal area. We hypothesized that the neuro-vascular encasement of structures located at the upper dorsal midbrain by the lipoma caused the symptoms. A review of the current literature of quadrigeminal lipoma cases with presenting symptoms is provided. Lipoma in quardrigeminal area could give rise to symptoms of psychosis.

  4. Catatonic schizophrenia: an international comparative study. (United States)

    Chandrasena, R


    Thirty-five hospitalized catatonic schizophrenic patients from Sri Lanka were compared with 22 patients in the U.K. and 13 in Canada. The phenomenology was established using the Present State Examination. Results suggest that ethnicity, chronicity of illness and reception of neuroleptic treatment may influence the lower prevalence of catatonic symptoms among the U.K. and Canadian schizophrenics. Onset of illness appears to be among young adults and mutism, stupor, mannerisms, stereotypes and negativism were the common catatonic symptoms observed.

  5. [Crossed aphasia: description of a case]. (United States)

    Rodríguez Campello, A; Pascual Calvet, J; Munteis, E; Gomis, M; Serra, A; Pou, A


    We describe a new case of crossed aphasia in a right-handed patient with a right hemispheric lesion. A right-handed man, 76 year-old, developed a sudden left hemiparesis with sensitive impairment and mutism. He has neither family history of left handeness or ambidexterity or vascular risk factors. CT cerebral scan showed a large infarct of the middle cerebral artery on the right side, with haemorrhagic suffusion. Cerebral MRI and EEG-cartography confirmed the indemnity of the left hemisphere. Aphasia studies confirmed a mutism with spared verbal comprehension, but alexia was present. A year later, left hemiparesis was recovered but aphasia remained. Crossed aphasia is rarely seen. It is caused by a right hemispheric lesion in right-handed subjects. Fluency is most commonly impaired. At onset, mutism is the common symptom, which evolves to expressive aphasia. Several hypothesis have been raised about the possible mechanisms involved. The few number of PET or SPECT studies performed in these patients have disclosed extensive areas of hypometabolism in the right hemisphere, that exceed the size of the image observed with CT scan or MRI.

  6. Dobutamine stress echocardiographyin distinguishing ischemic from nonischemic dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Miloradović Vladimir


    Full Text Available Introduction The aim of this study was to evaluate the diagnostic accuracy of dobutamine stress echocardiography for detection of coronary artery disease in patients with dilated cardiomyopathy. Detection of regional wall motion abnormalities at rest does not reliably distinguish ischemic from nonischemic cardiomyopathy. Material and methods To distinguish between ischemic and nonischemic dilated cardiomyopathy (DCM, we studied 50 patients with left ventricular dysfunction (20 ischemic and 30 nonischemic, detected by coronary angiography using dobutamine stress echocardiography. Echocardiographic images were obtained at baseline, low and paek dose of dobutamine. Rest and stress left ventricular wall motion scores were derived from analysis of regional wall motion. Results Dobutamine infusion was terminated after achievement of the target heart rate or maximal protocol dose in 16 (80% patients with ischemic heart disease and in 23 (73.3% patients with nonischemic heart disease. At rest, there were more normal segments (p<0.001 and a trend toward more akinetic segments (p, not significant per ischemic than per nonischemic DCM patients. However, either at rest or with low-dose dobutamine, individual data largely overlapped. At peak dose, in ischemic DCM, regional contraction worsened in many normal or dyssinergic regions at rest (in some cases after inprovement with low-dose dobutamine; in contrast, in nonischemic DCM, further mild impovement was observed in a variable number of left ventricular areas. Thus, with peak-dose dobutamine, more akinetic and less normal segments were present per ishemic than per nonischemic DCM patient (both, p<0.001. A value of six or more akinetic segments was 90% sensitive and 98% specific for ischemic DCM. Conclusions Our data show that analysis of regional contraction by dobutamine stress echocardiography can distinguish between.

  7. Phylogenomic Methods to Guide Paleontological Searches for the Early Cyanobacteria (United States)

    Blank, C. E.


    Phylogenomic methods can help paleontologists target their searches for early microbial microfossils and potentially help them better interpret the early fossil record. In this study, the deep-branching relationships in the cyanobacteria were resolved using whole genome sequences, multiple genes for taxa lacking genomes, and intein presence/absence in the DnaE protein. Once a framework tree was produced, characters were mapped onto the tree. Characters included morphology (unicellular vs. filamentous), habitat (marine vs. freshwater), metabolism (use of sulfide as electron donor, nitrogen fixation), presence/absence of complex morphological traits (akinetes, heterocysts, hormogonia), salt tolerance, and thermal tolerance. It was found that the earliest cyanobacteria were unicellular coccoids, with cell diameters cyanobacteria to freshwater deposits (lakes, streams) and to small diameter coccoids (not mats, not filaments). The earliest "cyanobacterial" microfossils (Eosynechococcus and Eoentophysalis) are large-diameter coccoids found in shallow marine platform carbonates. Because these cells have large diameters, if they were cyanobacteria one would also expect to see their sister taxa in the fossil record (i.e., large-diameter filamentous forms with sheaths, also akinetes). Because these are not found until 2.0 Ga (and akinetes until 1.5 Ga), this suggests that these earliest microfossils are not cyanobacteria. There are several instances in the cyanobacterial tree where ancestors with low salt tolerance gave rise to lineages that grow in brackish, marine, and/or hypersaline environments. This suggests that either the cyanobacteria first originated on continents and later colonized more saline environments, or that the cyanobacteria first originated in shallow "seas" that were not very saline but gradually became more saline by about 2.0 Ga. Because the continents were likely harsh environments (due to lack of an ozone layer and increased chemical and physical


    Institute of Scientific and Technical Information of China (English)

    吴忠兴; 虞功亮; 施军琼; 李仁辉


    Taxonomy of Aphanizomenon has been problematic for a long time due to the fact that the morphylogical characters of the filaments, vegetable cells, terminal cells, heterocysts, and akinetes in the genus are found to be variable. Based on the existing taxonomic system, Aphanizomenon strains collected in China were examined for their morphylogical charect-ers including filaments, vegetable cells, terminal cells, heterocysts, and akinetes. The results showed that two newly recorded species of Aphanizomen were found from Chinese freshwater bodies. Therefore, these two species including A. gracile Lemmermann and A. issatschenkoi( Usa(c)ev) Pro(s)kina-Lavrenko, were reported and described in this study for details.%由于束丝藻属(Aphanizomenon Mort.ex Flah.)的藻丝、营养细胞、藻丝末端细胞(Terminal cells)、异型胞(Heterocysts)、厚壁休眠孢子(Akinetes)的形态和大小等特征易变,对鉴定工作造成许多闲难.所以该属的分类一直以来是藻类学者面临的长期难题.基于当今束丝藻属的分类研究,对我国淡水水体束丝藻属进行了研究,比较了该属的藻丝、营养细胞、异形胞、厚壁孢子及末端细胞的特征,发现了我困淡水水体的2个新记录种:柔细束丝藻(Aphanizomenon gracile Lemmermann)和依沙束丝藻(A.issatschenkoi(Usacev)Progkina-Lavrenko),并对其形态特征进行了详细描述.

  9. Annual Gaseous Electronics Conference (37th) Held at Boulder, Colorado on 9-12 October 1984. Program and Abstracts. (United States)


    and J.M. Wadehra, AFWAL/APL, Wright- Ellena, IREQ,/arennes, Quebec, Canada Patterson AFB A-2 MOBILITY OF POSITIVE IONS IN SF6 B-6 DEVELOPMENT OF A...KINETIC INVESTIGATION OF STREAMER DEVELOPMENT: E.E. Kunhardt, Polytechnic Nea Uited TechnoLgy Inst. of New York, and Y. Tzeng, Auburn L. Newman...and 1.5 inches, respectively and a length of 4 inches. The gas flow was varied between 25 and 200 cm3s-1. The ion mobility evaluated from the current

  10. Occurrence and trends of selected nutrients, other chemical constituents, diatoms, and cyanobacteria in bottom sediment, Lake Maxinkuckee, northern Indiana (United States)

    Juracek, Kyle E.


    Bottom-sediment cores collected in 2013 were used to investigate the recent and predevelopment (pre-1863) occurrence of selected nutrients (total nitrogen and total phosphorus), carbon, 39 trace elements, diatoms, cyanobacterial akinetes, and 3 radionuclides in the bottom sediment of Lake Maxinkuckee, a kettle lake in northern Indiana. Total nitrogen concentrations in the recent sediment (since about 1970) were variable with no consistent trend indicated. Total phosphorus concentrations in the recent sediment generally were uniform from about 1970 to about 2000 and indicated consistent inputs to the lake during that time. Subsequently, the history of total phosphorus deposition apparently was obscured by postdepositional upward diffusion.

  11. Consultation Dilemma Catatonia in a Patient with Prior TBI: MentaI or Medical Disorder? (United States)

    Khalafian, Andrey; Dukes, Charles; Tucker, Phebe


    Mr. R, a 27 year old Hispanic male with history of traumatic brain injury (TBI) over ten years prior but no psychiatric history, presents to the psychiatric consultation service with recent onset of mutism, psychotic behavior and new diagnosis of epilepsy. The differential diagnosis is broad and includes both medical and psychiatric causes: post-ictal state, non-convulsive status epilepticus, delirium due to metabolic conditions, drugs, catatonia, conversion disorder, major depression with psychotic features, new onset schizophrenia or a combination of these possible diagnoses. We explore different medical causes that can present with symptoms of catatonia, as it is crucial to rule out a possible treatable medical cause.

  12. Malignant Catatonia

    Directory of Open Access Journals (Sweden)

    Ayca Ozkul


    Full Text Available Catatonia is a syndrome characterized by mutism, immobility, negativism, stereotypy, mannerisms, echophenomena, perseveration and passive obedience. The underlying causes can be psychiatric or may be associated with general medical status or neurological diseases. Additionally catatonia has two subtypes as malignant and nonmalignant catatonia. Main symptoms of malignant catatonia are hyperthermia and autonomic symptoms such as tachycardia, tachypnea and hyperhidrosis. It is important to make the diagnosis as early as possible for an appropriate medical treatment. Clinicians should be aware of the fatal outcome of the disease.

  13. Catatonia and autism: a historical review, with implications for electroconvulsive therapy. (United States)

    Dhossche, Dirk M; Reti, Irving M; Wachtel, Lee E


    Current autism research is historically separated from catatonia and other childhood psychotic disorders, although catatonia and autism share several common symptoms (mutism, echolalia, stereotypic speech and repetitive behaviors, posturing, grimacing, rigidity, mannerisms, and purposeless agitation). Electroconvulsive therapy (ECT) effectively treats catatonia and catatonia-related conditions of intractable compulsions, tics, and self-injury in people with autism. We assess the incidence of catatonic symptoms in autism, examine emerging ECT indications in people with autism and related developmental disorders, and encourage ethical debate and legal-administrative action to assure equal access to ECT for people with autism.

  14. Austistic Children: Bodily Factors in the Use of Language. (United States)

    Rhode, Maria


    This paper addresses factors other than symbolic capacity that can influence the use of language by children on the autism spectrum. Chief among the issues considered are the influence of bodily experience on the articulation of words and the influence of fantasies concerning bodily relationships on the construction of words and sentences. It is suggested that such considerations may shed light on the behavior of those children on the autism spectrum whose symbolic capacity is greater than might be assumed from their use of language, and also on that of some children with selective mutism.

  15. Historical note: Jules Séglas on language in dementia. (United States)

    Obler, L K; Albert, M L


    In his book Des troubles du language chez les aliénés (1892) Séglas applied the model of language processing of his teacher, Charcot, to analyze the language disorders of various groups of mentally ill, including patients considered to suffer from dementia. Among the language phenomena he described from dementia are logorrhea, neologisms, embolalia, near-mutism, angophrasia, automatic speech, alexia, and agraphia. Séglas must be credited for his early descriptions of how language behaviors cluster in various psychiatric and dementing diseases.

  16. Clinical and imaging characteristics of 16 patients with autoimmune neuronal synaptic encephalitis

    Directory of Open Access Journals (Sweden)

    N Kamble


    Conclusions: All patients with anti-VGKC, anti-NMDA and anti-TPO antibody positivity presented with a triad of behavioral changes, impaired cognition and seizures. Mutism was a predominant symptom in patients with an anti-NMDA antibody positivity, which may help in the early identification of this disorder. MRI brain showed changes restricted to limbic structures in anti-NMDA and anti-VGKC antibody positivity. An early diagnosis and treatment of autoimmune encephalitis is essential for a better outcome and for prevention of long-term sequel.

  17. Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis in a young Lebanese girl. (United States)

    Safadieh, Layal; Dabbagh, Omar


    Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a recently recognized autoimmune neurologic disorder that presents with severe neuropsychiatric symptoms in previously healthy children. A 4-year-old Lebanese girl presented with new-onset behavioral changes, orofacial dyskinesias, fluctuation in consciousness, inability to walk, and mutism. Antibodies directed against NMDA receptors were detected in the patient's serum and cerebrospinal fluid. Prompt treatment with a single course of intravenous immunoglobulin resulted in early complete recovery. This is the first case report of a Middle Eastern child affected with this condition.

  18. Potential for intracranial movements in pterosaurs. (United States)

    Prondvai, Edina; Osi, Attila


    Based on comparative anatomical, morphological, and phylogenetic considerations the potential of pterosaurs for cranial kinesis is assessed. Our investigation shows that whereas skeletally mature derived pterodactyloids have completely fused, rigid and doubtlessly akinetic skulls, skeletally immature derived pterodactyloids and more basal pterosaurs possess key features in the morphology of their otic and basal joints that are suggestive of cranial kinesis, namely streptostyly. In addition, pterosaurs exhibit an evolutionarily informative trend in the degree of cranial ossification, where it is low in most nonpterodactyloids (here named bifenestratans), intermediate in Rhamphorhynchus and Archaeopterodactyloidea, and high in derived pterodactyloids. Incomplete fusion could also indicate loose connections between skull elements. However, another crucial anatomical requirement of a kinetic skull, the permissive kinematic linkage is absent in all pterosaurian taxa. The fact, that the presence of permissive kinematic linkages in the skull is also a prerequisite of all types of cranial kinesis, provides hard evidence that all members of Pterosauria had akinetic skulls. Thus, the presence of the morphological attributes indicative of intracranial movements in some pterosaurs must be explained on grounds other than real potential for cranial kinesis. It could either be of mechanical or ontogenetic importance, or both. Alternatively, it might be considered as the morphological remnant of a real, kinetic skull possessed by the diapsid ancestors of pterosaurs.

  19. Pedunculopontine Nucleus Region Deep Brain Stimulation in Parkinson Disease: Surgical Anatomy and Terminology (United States)

    Hamani, Clement; Aziz, Tipu; Bloem, Bastiaan R.; Brown, Peter; Chabardes, Stephan; Coyne, Terry; Foote, Kelly; Garcia-Rill, Edgar; Hirsch, Etienne C.; Lozano, Andres M.; Mazzone, Paolo A.M.; Okun, Michael S.; Hutchison, William; Silburn, Peter; Zrinzo, Ludvic; Alam, Mesbah; Goetz, Laurent; Pereira, Erlick; Rughani, Anand; Thevathasan, Wesley; Moro, Elena; Krauss, Joachim K.


    Several lines of evidence over the last few years have been important in ascertaining that the pedunculopontine nucleus (PPN) region could be considered as a potential target for deep brain stimulation (DBS) to treat freezing and other problems as part of a spectrum of gait disorders in Parkinson disease and other akinetic movement disorders. Since the introduction of PPN DBS, a variety of clinical studies have been published. Most indicate improvements in freezing and falls in patients who are severely affected by these problems. The results across patients, however, have been variable, perhaps reflecting patient selection, heterogeneity in target selection and differences in surgical methodology and stimulation settings. Here we outline both the accumulated knowledge and the domains of uncertainty in surgical anatomy and terminology. Specific topics were assigned to groups of experts, and this work was accumulated and reviewed by the executive committee of the working group. Areas of disagreement were discussed and modified accordingly until a consensus could be reached. We demonstrate that both the anatomy and the functional role of the PPN region need further study. The borders of the PPN and of adjacent nuclei differ when different brainstem atlases and atlas slices are compared. It is difficult to delineate precisely the PPN pars dissipata from the nucleus cuneiformis, as these structures partially overlap. This lack of clarity contributes to the difficulty in targeting and determining the exact localization of the electrodes implanted in patients with akinetic gait disorders. Future clinical studies need to consider these issues. PMID:27723662

  20. Fungal parasitism: life cycle, dynamics and impact on cyanobacterial blooms.

    Directory of Open Access Journals (Sweden)

    Mélanie Gerphagnon

    Full Text Available Many species of phytoplankton are susceptible to parasitism by fungi from the phylum Chytridiomycota (i.e. chytrids. However, few studies have reported the effects of fungal parasites on filamentous cyanobacterial blooms. To investigate the missing components of bloom ecosystems, we examined an entire field bloom of the cyanobacterium Anabaena macrospora for evidence of chytrid infection in a productive freshwater lake, using a high resolution sampling strategy. A. macrospora was infected by two species of the genus Rhizosiphon which have similar life cycles but differed in their infective regimes depending on the cellular niches offered by their host. R. crassum infected both vegetative cells and akinetes while R. akinetum infected only akinetes. A tentative reconstruction of the developmental stages suggested that the life cycle of R. crassum was completed in about 3 days. The infection affected 6% of total cells (and 4% of akinètes, spread over a maximum of 17% of the filaments of cyanobacteria, in which 60% of the cells could be parasitized. Furthermore, chytrids may reduce the length of filaments of Anabaena macrospora significantly by "mechanistic fragmentation" following infection. All these results suggest that chytrid parasitism is one of the driving factors involved in the decline of a cyanobacteria blooms, by direct mortality of parasitized cells and indirectly by the mechanistic fragmentation, which could weaken the resistance of A. macrospora to grazing.

  1. A tautology in the classification of generalized non-convulsive epileptic seizures. (United States)

    Seino, M; Fujiwara, T; Miyakoshi, M; Yagi, K


    Five patients with the Lennox-Gastaut syndrome who have shown generalized nonconvulsive seizures were presented. The seizure manifestations which occurred spontaneously were documented by simultaneous recording and analyzed in terms of clinical and electroencephalographic correlates. According to the diagnostic criteria of the International Classification, it was possible, on the one hand, to regard them as "atypical complex absences" in which the impairment of consciousness is accompanied by other symptoms, which tend to dominate the clinical picture. They were: hypotonic, hypertonic, myoclonic and akinetic components, respectively. On the other hand, if we give a special weight to the accompanying symptoms, it is entirely possible that they are at the same time diagnosed atonic, axial tonic, bilateral myoclonic and akinetic seizures. The initial impairment of consciousness is common to all the seizure manifestations, and the ictal and interictal EEG expressions are not of diagnostic significance. A question arises as to whether two different nomenclatures were arbitrarily given to a unique ictal manifestation or not as far as the generalized non-convulsive seizures were concerned.

  2. Diagnosis of a Strangulated Laparoscopic Incisional Hernia with Point-of-Care Ultrasonography

    Directory of Open Access Journals (Sweden)

    Argintaru, Niran


    Full Text Available The use of point-of-care ultrasound for the diagnosis of bowel obstructions and hernias is becoming increasingly common in the emergency department (ED. Using a relatively rare case of an incisional port hernia, we demonstrate the ultrasound findings of a strangulated hernia causing a partial small bowel obstruction. A 46-year-old female presented four days following a laparoscopic surgery complaining of abdominal pain, nausea and lack of bowel movements. There was a palpable mass in the left lower quadrant under the 12mm trocar port incision. ED point-of-care ultrasound revealed herniated akinetic loops of bowel through her laparoscopy incision. This is the first case report to describe the use of point-of-care ultrasound for the diagnosis of a strangulated incisional port hernia at the bedside. [West J Emerg Med. 2015;16(3:450–452.

  3. Pharmacological profile of the abeorphine 201-678, a potent orally active and long lasting dopamine agonist

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    Jaton, A.L.; Giger, R.K.A.; Vigouret, J.M.; Enz, A.; Frick, W.; Closse, A.; Markstein, R.


    The central dopaminergic effects of an abeorphine derivative 201-678 were compared to those of apomorphine and bromocriptine in different model systems. After oral administration, this compound induced contralateral turning in rats with 6-hydroxydopamine induced nigral lesions and exhibited strong anti-akinetic properties in rats with 6-hydroxydopamine induced hypothalamic lesions. It decreased dopamine metabolism in striatum and cortex, but did not modify noradrenaline and serotonin metabolism in the rat brain. 201-678 counteracted the in vivo increase of tyrosine hydroxylase activity induced by ..gamma..-butyrolactone. In vitro it stimulated DA-sensitive adenylate cyclase and inhibited acetylcholine release from rat striatal slices. This compound had high affinity for /sup 3/H-dopamine and /sup 3/H-clonidine binding sites. These results indicate that 201-678 is a potent, orally active dopamine agonist with a long duration of action. Furthermore it appears more selective than other dopaminergic drugs. 29 references, 5 figures, 3 tables.

  4. Repeated renal infarction in native and transplanted kidneys due to left ventricular thrombus formation caused by antiphospholipid antibody syndrome. (United States)

    Scully, Paul; Leckstroem, Daniel C; McGrath, Andrew; Chambers, John; Goldsmith, David J


    Antiphospholipid syndrome can be a feature of several underlying conditions, such as lupus, but it can also occur idiopathically. Diagnosis usually comes after investigation of recurrent venous or arterial thromboses, emboli, or hypertension/proteinuria where the kidney is involved and is usually confirmed by laboratory testing. We describe a case of a man with a myocardial infarction who developed mural thrombus in an akinetic left ventricular segment but then who recurrently embolized first to one of his native kidneys and then later to a transplanted kidney. Although the clinical behavior was typical of antiphospholipid syndrome, it took numerous laboratory assays over many years until finally the problem was confirmed and life-long warfarin therapy instituted.

  5. Stress-induced cardiomyopathy (Takotsubo)--broken heart and mind? (United States)

    Redfors, Björn; Shao, Yangzhen; Omerovic, Elmir


    Stress-induced cardiomyopathy (SIC), also known as Takotsubo cardiomyopathy, is characterized by severe but potentially reversible regional left ventricular wall motion abnormalities, ie, akinesia, in the absence of explanatory angiographic evidence of a coronary occlusion. The typical pattern is that of an akinetic apex with preserved contractions in the base, but other variants are also common, including basal or midmyocardial akinesia with preserved apical function. The pathophysiology of SIC remains largely unknown but catecholamines are believed to play a pivotal role. The diverse array of triggering events that have been linked to SIC are arbitrarily categorized as either emotional or somatic stressors. These categories can be considered as different elements of a continuous spectrum, linked through the interface of neurology and psychiatry. This paper reviews our current knowledge of SIC, with focus on the intimate relationship between the brain and the heart.

  6. Parkinson's disease severity and motor subtype influence physical capacity components

    Directory of Open Access Journals (Sweden)

    Marcelo Pinto Pereira


    Full Text Available The severity of Parkinson's disease (PD and PD's motor subtypes influence the components of physical capacity. The aim of this study was to investigate the impact of both PD severity and motor subtype in the performance of these components. Thirty-six PD patients were assigned into four groups: Tremor (TD initial and TD mild, akinetic-rigid (AR initial, and AR mild. Patients' strength, balance, coordination, mobility and aerobic capacity were evaluated and groups were compared using a two-way ANOVA (severity and subtype as factors. AR presents a poorer performance than TD in almost all tests. Also this performance was worsened with the advance of the disease in AR, contrary to TD. We conclude that AR and TD subgroups are different about their performance on physical capacity components, moreover, this performance worsens with the advance of the disease of the AR group, but not for TD.

  7. Clinical features of progressive supranuclear palsy in 105 Chinese patients

    Institute of Scientific and Technical Information of China (English)

    Jing Hou; Ruibiao Guo; Tong Chen; Xiaohong Zhang; Weiping Wu; Zhenfu Wang


    OBJECTIVE: To thoroughly investigate clinical characteristics of progressive supranuclear palsy (PSP) in a Chinese population.METHODS: Computer-based online searches through China National Knowledge Infrastructure and Weipu Periodical Database were performed to collect case reports of PSP published between 1980 and 2009. Clinical characteristics were analyzed.RESULTS: A total of 58 studies comprising 105 patients (76 males and 29 females) were included. All cases were sporadic and free of family history. The mean age at onset was 60.6 ± 9.1 years, and the mean course from onset of symptoms to diagnosis was 3.4 ± 2.4 years. The male-to-female ratio was approximately 3: 1. Onset was characterized by akinetic-rigid features and accounted for 34.3% of all cases, followed by early postural instability (25.5%), pseudobulbar palsy (9.8%), cognitive impairment (9.8%), and vertical supranuclear ophthalmoplegia (7.8%). With disease progression, vertical supranuclear ophthalmoplegia was reported in 95.1% of cases, followed by akinetic-rigid features (83.3%), pseudobulbar palsy (82.4%), axial dystonia (75.5%), cognitive impairment (72.5%), and early postural instability (69.6%). A total of 70.5% of patients exhibited abnormal electroencephalograms, and 21.4% exhibited mild abnormalities in cerebrospinal fluid. Brain CT scanning results of 37 patients showed 37.8% with midbrain and concurrent cerebral hemisphere atrophy, and 5.4% and 24.3% with midbrain and cerebral hemisphere atrophy, respectively. Brain MRI scanning results of 55 patients revealed a total of 16.4% patients with midbrain atrophy, 23.6% with midbrain and concurrent cerebral hemisphere atrophy, 32.7% with cerebral hemisphere atrophy, and 11% with brainstem atrophy. The percentage of midbrain atrophy revealed by MRI was greater than by CT. All 11 patients subjected to Mini-Mental State Examination scored < 23. A total of 10 patients underwent brain electrophysiological examination, and 80% presented with

  8. [Segmental wall movement of the left ventricle in healthy persons and myocardial infarct patients studied by a catheter-less nuclear medical method (camera-cinematography of the heart)]. (United States)

    Geffers, H; Sigel, H; Bitter, F; Kampmann, H; Stauch, M; Adam, W E


    Camera-Kinematography is a nearly noninvasive method to investigate regional motion of the myocard, and allows evaluation of the function of the heart. About 20 min after injection of 15-20 mCi of 99mTC-Human-Serum-Albumin, when the tracer is distributed homogenously within the bloodpool, data acquisition starts. Myocardial wall motion is represented in an appropriate quasi three-dimensional form. In this representation scars can be revealed as "silent" (akinetic) regions, aneurysms by asynchronic motion. Time activity curves for arbitrarily chosen regions can be calculated and give an equivalent for regional volume changes. 16 patients with an old infarction have been investigated. In fourteen cases the location and extent of regions with abnormal motion could be evaluated. Only two cases of a small posterior wall infarction did not show deviations from normal contraction pattern.

  9. Transient stress cardiomyopathies in the elderly: Clinical & Pathophysiologic considerations

    Institute of Scientific and Technical Information of China (English)

    Michael A Chen


    Transient stress-induced cardiomyopathies have been increasingly recognized and while rare,they tend to affect elderly women more than other demographic groups.One type,often called tako-tsubo cardiomyopathy (TTC),is typically triggered by significant emotional or physical stress and is associated with chest pain,electrocardiogram (ECG) changes and abnormal cardiac enzymes.Significant left ventricular regional wall motion abnormalities usually include an akinetic "ballooning" apex with normal or hyperdynamic function of the base.A second type,often called neurogenic stunned myocardium,typically associated with subarachnoid hemorrhage,also usually presents with ECG changes and positive enzymes,but the typical wall motion abnormalities seen include normal basal and apical left ventricular contraction with akinesis of the mid-cavity in a circumferential fashion.The pathophysiology,clinical care and typical courses,are reviewed.

  10. Sedimentation and occurrence and trends of selected nutrients, other chemical constituents, and cyanobacteria in bottom sediment, Clinton Lake, northeast Kansas, 1977-2009 (United States)

    Juracek, Kyle E.


    A combination of available bathymetric-survey information and bottom-sediment coring was used to investigate sedimentation and the occurrence of selected nutrients (total nitrogen and total phosphorus), organic and total carbon, 25 trace elements, cyanobacterial akinetes, and the radionuclide cesium-137 in the bottom sediment of Clinton Lake, northeast Kansas. The total estimated volume and mass of bottom sediment deposited from 1977 through 2009 in the conservation (multi-purpose) pool of the reservoir was 438 million cubic feet and 18 billion pounds, respectively. The estimated sediment volume occupied about 8 percent of the conservation-pool, water-storage capacity of the reservoir. Sedimentation in the conservation pool has occurred about 70 percent faster than originally projected at the time the reservoir was completed. Water-storage capacity in the conservation pool has been lost to sedimentation at a rate of about 0.25 percent annually. Mean annual net sediment deposition since 1977 in the conservation pool of the reservoir was estimated to be 563 million pounds per year. Mean annual net sediment yield from the Clinton Lake Basin was estimated to be 1.5 million pounds per square mile per year. Typically, the bottom sediment sampled in Clinton Lake was at least 99 percent silt and clay. The mean annual net loads of total nitrogen and total phosphorus deposited in the bottom sediment of Clinton Lake were estimated to be 1.29 million pounds per year and 556,000 pounds per year, respectively. The estimated mean annual net yields of total nitrogen and total phosphorus from the Clinton Lake Basin were 3,510 pounds per square mile per year and 1,510 pounds per square mile per year, respectively. Throughout the history of Clinton Lake, total nitrogen concentrations in the deposited sediment generally were uniform and indicated consistent inputs to the reservoir over time. Likewise, total phosphorus concentrations in the deposited sediment generally were uniform

  11. [Nonlinear dynamics of involuntary shaking of the human hand under motor dysfunction]. (United States)

    Dick, O E; Nozdrachev, A D


    Using nonlinear dynamic methods we examined wavelet and multifractal features of involuntary shaking (tremor) arising during the performance the motor task (sustaining effort of fingers under isometric conditions). The wavelet score (the maximum of the global wavelet spectrum) and multifractal parameters (the width and asymmetry of the singularity spectrum) significantly differ in tremor of healthy subjects and patients with akinetic-rigid form of Parkinson's disease. The relations between the change of the patient state connected with the drug relief of parkinsonian symptoms and the variations of the parameter values have been obtained. The suggested analytic approach for noninvasive study of integrative activity of the central nervous system, formed as the motor exit during realization of the motor task, enables not only to estimate quantitatively the degree of deviation of the motor function from the healthy one, but it can help to a clinician to choose the optimal treatment in every particular case.

  12. Ecological distribution of harmful epiphytic Oscillatoriales in Alexandria coast, Egypt, with special reference to DNA identification

    Institute of Scientific and Technical Information of China (English)

    Amany Abdel Hamid Ismael; Eman Abdel Razak Mohamed; Mostafa Mohamed El-Sheikh; Wafaa Hassan Hegazy


    Objective: To identify the potentially harmful epiphytic Oscillatoriales species and follow up their distribution along Alexandria coast. Methods: Samples were collected bimonthly from April 2009 to February 2010 at three sites along Alexandria coast. Both morphological and molecular analyses were used for identifying the dominant species.Results:Five species belonging to two families were identified; Oscillatoria acutissima, Oscillatoria nigroviridis, Oscillatoria sp., Lyngbya majuscule and Phormidium formosum. Their cell density ranged from 103 to 126X103 filament g-1 fresh weight macroalgae. The morphological study of the dominant species, Oscillatoria sp. (Oscillatoria sp. W1) showed much similarity withPlanktothrix agardhii with no heterocysts and akinetes, while molecular ananlysis (16S rDNA) clustered the species in the same group with Anabaena sp.Conclusions:The 16S rDNA genes are not suitable for identifying Oscillatoriales during the present study and another molecular method should be used instead.

  13. Paleobiology of the Mesoproterozoic Billyakh Group, Anabar Uplift, northern Siberia (United States)

    Sergeev, V. N.; Knoll, A. H.; Grotzinger, J. P.


    Silicified peritidal carbonates of the Mesoproterozoic Kotuikan and Yusmastakh Formations, Anabar Uplift, northeastern Siberia, contain exceptionally well-preserved microfossils. The assemblage is dominated by ellipsoidal akinetes of nostocalean cyanobacteria (Archaeoellipsoides) and problematic spheroidal unicells (Myxococcoides); both are allochthonous and presumably planktonic. The assemblage also includes distinctive mat-forming scytonematacean and entophysalidacean cyanobacteria, diverse short trichomes interpreted as cyanobacterial hormogonia or germinated akinetes, rare longer trichomes, and several types of colonial unicells. Although many taxa in the Kotuikan-Yusmastakh assemblage are long-ranging prokaryotes, the overall character of the assemblage is distinctly Mesoproterozoic, with its major features shared by broadly coeval floras from Canada, China, India, and elsewhere in Siberia. Microfossils also occur in middle to inner shelf shales of the Ust'-Il'ya and lower Kotuikan Formations. Leiosphaerid acritarchs (up to several hundred microns in diameter) characterize this facies. As in other Mesoproterozoic acritarch assemblages, acanthomorphic and other complex forms that typify Neoproterozoic assemblages are absent. The combination in Billyakh assemblages of exceptional preservation and low eukaryotic diversity supports the hypothesis that nucleated organisms diversified markedly near the Mesoproterozoic-Neoproterozoic boundary. The assemblages also demonstrate the antiquity of cyanobacteria capable of cell differentiation and suggest the importance of both changing peritidal substrates and evolving eukaryotes in determining stratigraphic patterns of Proterozoic prokaryotes. The permineralized assemblage contains 33 species belonging to 17 genera. Ten new species or new combinations are proposed: Archaeoellipsoides costatus n. sp., A. elongatus n. comb., A. dolichos n. comb., A. minor n. nom., A. crassus n. comb., A. major n. comb., A. bactroformis n

  14. A prospective study of freezing of gait with early Parkinson disease in Chinese patients. (United States)

    Zhang, Hongbo; Yin, Xifan; Ouyang, Zhiyuan; Chen, Jing; Zhou, Shenghua; Zhang, Changguo; Pan, Xin; Wang, Shiliang; Yang, Junxiang; Feng, Yaoyao; Yu, Ping; Zhang, Qiangchun


    This study investigated the risk factors for freezing of gait (FOG) in the early stage of Parkinson disease in China, using a sample of 248 patients who were followed for 3 years. Part III of the Unified Parkinson Disease Rating Scale and the modified Hoehn-Yahr grading scale were used to evaluate the severity of motor symptoms. Nonmotor symptoms were assessed using the Hamilton Anxiety Rating Scale, Hamilton Depression Rating Scale (HAMD), and Non-Motor Symptoms Scale (NMSS). The end-point was the presence of FOG at the end of follow-up; patients with FOG were classified as freezers. The risk factors for FOG were analyzed at the end of the first, second, and third years after baseline. There were 40 freezers (16.13%) 1 year later, 98 (39.52%) 2 years later, and 128 (51.61%) 3 years later. FOG 3 years later was associated with the following variables: depression (P = 0.003), older age, living in the countryside, lower education, akinetic-rigid style, lower limbs as site of onset, early use of levodopa, higher daily dose of levodopa, and not using amantadine or selegiline and dopamine receptor agonists (P Parkinson disease were more likely to experience FOG if: they were older, or from the countryside; had an akinetic-rigid style, anxiety, or higher NMSS scores; they used levodopa early or did not use amantadine or selegiline; their lower limbs were the site of onset; or they had more severe motor disability or higher HAMD scores at baseline.

  15. Adult onset pigmentary orthochromatic leukodystrophy with ovarian dysgenesis. (United States)

    Verghese, J; Weidenheim, K; Malik, S; Rapin, I


    Pigmentary type of orthochromatic leukodystrophy (POLD) is an adult-onset leukodystrophy, characterized pathologically by the presence of glial and microglial cytoplasmic pigment inclusions. The complete phenotype, genotype and pathogenetic mechanisms in POLD have not been elucidated. We followed for 18 years a woman with autopsy-proven POLD, who presented with 'frontal' dementia and spasticity. Her further course was marked by progressive mutism, apraxia and seizures. Her sister had died of the same disease after a much more rapidly progressing course. These sisters had primary infertility with pathologic evidence of streak ovaries. Diagnosis was confirmed in both cases by post-mortem examination. POLD is a rare cause of adult-onset leukodystrophy presenting with dementia. Ovarian dysgenesis is extremely rare in the absence of demonstrable chromosomal abnormalities and extends the clinical spectrum of POLD.

  16. [Study of literature on disease in the Rongchengshi of the Warring States]. (United States)

    Luo, Bao-Zhen


    Many scholars have studied and disputed over the names of diseases recorded in Rongchengshi, bamboo slips of the Chu kingdon in the Warring States collected in the Shanghai Museum. According to comments of scholars and other literature it could be concluded: JinLong in slip 2 and AnLong in slip 36 means deaf-mutism; MaoFa in slip 2 means blindness; PiBi in slip 2 means lameness; ZhangZhe in slip 2 means persons who are strong and tall; An in slip 3 that it is the variant form of Ying, which means thyroid enlargement; Lou in slip 2 it means kyphosis of the spine; Xia in slip 3 it means pruritus and scabies; Δ in slip 36 means blindness; and Shang in slip 37 means bald head.


    Directory of Open Access Journals (Sweden)

    Pavan Kumar


    Full Text Available Marfan syndrome is a heritable condition that affects the connective tissues of Human body. It can affect any body systems including the skeleton, eyes, heart and blood vessels, nervous system, skin, and lungs. Estimates indicate that approximately 1 in 3,000 to 5,000 individuals have Marfan syndrome and most individuals with Marfan’s syndrome have another affected family member which are due to new genetic mutations. We present to you an interesting rare case which reported to our hospital few months back with features of Marfan’s syndrome. Without cardiac or respiratory involvement and associated Mutism and Hypogonadism. In the family no other member was affected.

  18. Avant-propos

    Directory of Open Access Journals (Sweden)

    Jacques-Emmanuel Bernard


    Full Text Available En évoquant les grottes de Lascaux, Fernand Braudel s’interrogeait : pourquoi, à l’heure où naît le premier art des hommes, la Méditerranée est-elle silencieuse en quelque sorte ? S’occuperait-elle d’autre chose ? Vivrait-elle autrement ? Le miracle y serait-il, en Orient, le développement décisif des langages parlés ? Le mutisme méditerranéen… On ne pourrait rêver plus beau paradoxe pour ouvrir un numéro de Babel sur les rhétoriques enfantées par la Mère Méditerranée- pour faire allusion...

  19. Multi-Modal Ultra-Widefield Imaging Features in Waardenburg Syndrome (United States)

    Choudhry, Netan; Rao, Rajesh C.


    Background Waardenburg syndrome is characterized by a group of features including; telecanthus, a broad nasal root, synophrys of the eyebrows, piedbaldism, heterochromia irides, and deaf-mutism. Hypopigmentation of the choroid is a unique feature of this condition examined with multi-modal Ultra-Widefield Imaging in this report. Material/Methods Report of a single case. Results Bilateral symmetric choroidal hypopigmentation was observed with hypoautofluorescence in the region of hypopigmentation. Fluorescein angiography revealed a normal vasculature, however a thickened choroid was seen on Enhanced-Depth Imaging Spectral-Domain OCT (EDI SD-OCT). Conclusion(s) Choroidal hypopigmentation is a unique feature of Waardenburg syndrome, which can be visualized with ultra-widefield fundus autofluorescence. The choroid may also be thickened in this condition and its thickness measured with EDI SD-OCT. PMID:26114849

  20. Renal Failure in Dementia with Lewy Bodies Presenting as Catatonia

    Directory of Open Access Journals (Sweden)

    Robert Fekete


    Full Text Available Catatonia, originally described by Karl Kahlbaum in 1874, may be regarded as a set of clinical features found in a subtype of schizophrenia, but the syndrome may also stem from organic causes including vascular parkinsonism, brain masses, globus pallidus lesions, metabolic derangements, and pharmacologic agents, especially first generation antipsychotics. Catatonia may include paratonia, waxy flexibility (cerea flexibilitas, stupor, mutism, echolalia, and catalepsy (abnormal posturing. A case of catatonia as a result of acute renal failure in a patient with dementia with Lewy bodies is described. This patient recovered after intravenous fluid administration and reinstitution of the atypical dopamine receptor blocking agent quetiapine, but benzodiazepines and amantadine are additional possible treatments. Recognition of organic causes of catatonia leads to timely treatment and resolution of the syndrome.

  1. Electroconvulsive therapy for catatonia in juvenile neuropsychiatric lupus. (United States)

    Leon, T; Aguirre, A; Pesce, C; Sanhueza, P; Toro, P


    Neuropsychiatric manifestations are serious and frequent complications of systemic lupus erythematous (SLE). Catatonia is a neuropsychiatric disorder characterized by motor disturbance (including waxy flexibility and catalepsy), stupor, excitement, negativism, mutism, echopraxia and echolalia. Catatonia associated with SLE has been only rarely reported, especially in children. Here we present a case of a 14-year-old patient encountered in consultation-liaison psychiatry who presented catatonia associated with SLE. Her catatonia was refractory to treatment with pulse methylprednisolone, intravenous cyclophosphamide and rituximab. The patient responded to a combined therapy of electroconvulsive therapy and benzodiazepines. The present case suggests that although rarely reported, catatonia seen in the background of SLE should be promptly identified and treated to reduce the morbidity.

  2. Malignant catatonia responsive to low doses of lorazepam: case report

    Directory of Open Access Journals (Sweden)

    Diego Fernando Moreira Matias

    Full Text Available Abstract CONTEXT: Catatonia can be divided into non-malignant or malignant. The latter is characterized by autonomic instability, exhibiting high fever, tachycardia and hypertension, and is regarded as a fulminant and rapidly progressive subtype. CASE REPORT: This article reports a case of malignant catatonia in a 43-year-old patient who had been presenting psychiatric disorders for the last three years. The patient was stable, maintaining mutism, immobility and autonomic abnormalities. Oral lorazepam (1 mg every eight hours was introduced and, in a few hours, the patient became afebrile. Two days later, the patient was already responding to verbal commands. CONCLUSIONS: Early intervention with lorazepam reduced the evolution of this patient to a fatal complication. Therefore, this case report sought to show that early diagnosis and intervention reduced the occurrence of serious and irreversible clinical outcomes.

  3. [Catatonia in the elderly]. (United States)

    Chiba, Yuhei; Odawara, Toshinari


    Catatonia is a syndrome characterized by mutism, stupor, immobility, negativism, posturing, stereotypy, and echophenomena. Not only patients with schizophrenia, but also patients with general medical disease, mood disorder, and substance-related disorder exhibit catatonia. In the patients with catatonia, it is recommended to examine whether they have a general medical disease. We present two catatonic elder patients. Case 1 exhibited catatonia with vascular dementia, and was revealed to have anti-phospholipid antibody syndrome. Case 2 exhibited catatonia with dementia with Lewy bodies, and was revealed to have Hashimoto's encephalopathy. The first recommended treatment for catatonia is benzodiazepines. In case of benzodiazepine resistance or malignant catatonia, it should be considered electroconvulsive therapy, but it needs to be carefully implemented for elder patients.

  4. [Aphasia of deep localization]. (United States)

    Saggese, J A; Toboada, E O; Duhart, J E; Adaro, F V


    A neurolinguistic and cerebral computed tomography (CT) study was carried out in 60 patients with aphasia. Fourteen had predominant subcortical involvement. Six of these showed involvement of the basal ganglia (5 with thalamic involvement), with small mass effect and small volume (less than 5 ml). The type of aphasia in this group was not uniform; it was remarkable, however, that initially there was mutism or initially non fluid language which soon became fluid in one half of the patients. Repetition and partially or totally preserved comprehension, together with dysarthic, dysprosodic and hypophonic abnormalities, were very common. The latter even had a more prolonged course than aphasic abnormalities. All the lesions in the remaining 8 patients had greater volume and were paracapsular in topography; 5 patients behaved as global aphasia (with a mean calculated volume of 23.7 ml), and the remaining 3 showed fluid aphasia, with an intermediate size (15 ml).

  5. Classic Pick's disease type with ubiquitin-positive and tau-negative inclusions: case report

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    Brito-Marques Paulo Roberto de


    Full Text Available We report on a patient presenting Pick's disease similar to the one reported by Pick in 1892, with ubiquitin-positive and tau-negative inclusions. His diagnosis was made on the basis of clinical (language disturbance and behavioural disorders, neuropsychological (progressive aphasia of the expression type and late mutism, neuroimaging with magnetic resonance (bilateral frontal and temporal lobes atrophy and brain single photon emission computed tomography (frontal and temporal lobes hypoperfusion studies. Macroscopic examination showed atrophy on the frontal and temporal lobes. The left hippocampus displayed a major circumscribed atrophy. The diagnostic confirmation was made by the neuropathological findings of the autopsy that showed neuronal loss with gliosis of the adjacent white matter and apearence of status spongiosus in the middle frontal and especially in the upper temporal lobes. There were also neuronal swelling (ballooned cell and argyrophilic inclusions (Pick's bodies in the left and right hippocampi. Anti-ubiquitin reaction tested positive and anti-tau tested negative.

  6. Post-partum transverse sinus thrombosis presenting as acute psychosis. (United States)

    Dhasmana, Devesh Janardan; Brockington, Ian F; Roberts, Ann


    Whilst cerebral vascular disease and mental illness in the post-partum period are well recognised, their co-existence and the concept of organic psychoses in pregnancy, parturition and the puerperium remains poorly appreciated (Brockington 2006; Brockington Arch Women's Ment Health 10: 177-178, 2007a; Brockington Arch Women's Ment Health 10: 305-306, b). We report a woman who was referred to the Medical team on-call with a mixed presentation of euphoria, mutism and aggressive behaviour but ultimately demonstrated to have a transverse sinus thrombosis and recovered well with anti-coagulation. This serves an important reminder of the implications of a missed medical diagnosis in this high-risk and vulnerable group of patients.

  7. Catatonia from its creation to DSM-V: Considerations for ICD. (United States)

    Fink, Max


    Catatonia was delineated only as a type of schizophrenia in the many American Psychiatric Association DSM classifications and revisions from 1952 until 1994 when "catatonia secondary to a medical condition" was added. Since the 1970s the diagnosis of catatonia has been clarified as a syndrome of rigidity, posturing, mutism, negativism, and other motor signs of acute onset. It is found in about 10% of psychiatric hospital admissions, in patients with depressed and manic mood states and in toxic states. It is quickly treatable to remission by benzodiazepines and by ECT. The DSM-V revision proposes catatonia in two major diagnostic classes, specifiers for 10 principal diagnoses, and deletion of the designation of schizophrenia, catatonic type. This complex recommendation serves no clinical or research purpose and confuses treatment options. Catatonia is best considered in the proposed ICD revision as a unique syndrome of multiple forms warranting a single unique defined class similar to that of delirium.

  8. [Anxiety disorders in DSM-5]. (United States)

    Márquez, Miguel


    The fifth edition of Diagnostic and Statistical Manual, the DSM-5 appeared officially in May 2013 during the development of the 166th Annual Meetingof the American Psychiatric Association (APA) in San Francisco. The drafting process was long and complex; much of the debate became public so that the expectations were great. And it must be said that the new edition did not disappoint, as many changes were made in relation to their predecessors. In Chapter of Anxiety Disorders, which is reviewed in this article, the changes were significant. Obsessive-compulsive disorder and Stress-related disorders were excluded and new clinical pictures, such as separation anxiety disorder and selective mutism, were included. And took place was the long awaited split between panic disorder and agoraphobia, now two separate disorders.

  9. 新疆地区不同临床分型帕金森病患者影响因素分析%The Influencing Factors for Parkinson' s Disease Patients with Different Clinical Type in Xinjiang

    Institute of Scientific and Technical Information of China (English)

    王艳艳; 阿克博塔·阿历戈伐; 杨新玲


    Objective To investigate the influencing factors for Parkinsion's disease( PD)patients with different clinical type in Xinjiang,to provide a theoretical basis for early prevention and treatment of disease. Methods By using the method of retrospective study,259 patients who were diagnosed with PD in the First Affiliated Hospital of Xinjiang Medical University from January 2004 to December 2013, were selected as study subjects. The age, gender, nationality, smoking history,drinking history,history of tea drinking,history of pesticide contact,positive family history,history of hypertension, history of diabetes,the degree of education,occupation and body mass index( BMI)of patients were investigated. Results 142 cases were classified as tremor type,90 cases were classified as akinetic - rigid type,27 cases were classified as mixed type. There were significant differences in age among tremor type PD patient group,akinetic-rigid type PD patient group and mixed type PD patient group(P﹤0. 05). The PD patients' clinical type with different nationality,smoking history,history of tea drinking,history of pesticide contact and occupation showed statistical differences( P﹤0. 05 ) . The patients' clinical type with different gender,drinking history,positive family history,history of hypertension,history of diabetes,education degree and BMI showed no statistical difference( P ﹥0. 05 ) . According to the results of multivariate logistic regression analysis, nationality,and history of tea drinking had a regressive relationship with tremor type,respectively;age and smoking history had a regressive relationship with akinetic - rigid type, respectively;nationality had a regressive relationship with mixed type, respectively(P﹤0. 05). Conclusion The most common PD types are tremor type and akinetic-rigid type,PD patients with different clinical type has different age,few tremor type PD patients are Han or tea drinkers,the most common type among old PD patients or smoker PD

  10. Transtorno de humor bipolar: diversas apresentações de uma mesma doença Bipolar mood disorder: different occurrences of the same disease

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    Audrey Regina Magalhães Braga


    Full Text Available O presente relato tem por objetivo acompanhar a evolução de um caso que se apresentou como mutismo seletivo e evoluiu para transtorno de humor bipolar e chamar atenção para a diversidade de sintomas possíveis no transtorno de humor. Em geral, o mutismo seletivo tem início na idade pré-escolar, porém os sintomas chamam mais atenção na idade escolar. A importância do diagnóstico e tratamento precoces reside na prevenção das complicações, tais como distúrbios no desenvolvimento social e acadêmico e na auto-estima, além da possibilidade de evolução para outros transtornos de ansiedade. O transtorno de humor bipolar é uma doença mental caracterizada por variações extremas no humor. Na criança, prejudica o crescimento emocional e seu desenvolvimento. É confundido com transtorno de déficit de atenção/hiperatividade e transtornos de comportamento, tais como transtorno de conduta e desafiador opositivo. A paciente do sexo feminino, quando avaliada, estava com 5 anos e 9 meses de idade. Concluiu-se o diagnóstico como mutismo seletivo. Foi indicada psicoterapia, com orientações aos pais e à escola, e iniciado o uso de inibidores seletivos de recaptação da serotonina, com boa resposta aos sintomas de ansiedade, mas passou a apresentar piora significativa do comportamento. Foi levantada a hipótese diagnóstica de transtorno de humor bipolar de início precoce. Medicada com estabilizador, apresentou adequação na terceira medicação e evolução satisfatória. O mutismo seletivo, considerado um transtorno de ansiedade na infância, pode ser pródromo para outros quadros psiquiátricos na infância. Há necessidade que os pediatras, médicos que primeiro acessam essas crianças, assim como os psiquiatras da infância, estejam atentos à riqueza de sintomas que pode dar seguimento ao quadro.The purpose of the present case report is to follow the evolution of a child who presented selective mutism and evolved into bipolar

  11. Diagnostic pitfalls in Parkinson's disease: case report Dificuldades diagnósticas na doença de Parkinson: relato de caso

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    Flávio Augusto Sekeff-Sallem


    Full Text Available Parkinson's disease (PD is characterized by resting tremor, rigidity and bradykinesia. In 80% of cases, the disorder begins with upper limb resting tremor. However, there are some presenting atypical features that make the diagnosis even more difficult and intriguing. The disorder can have its onset below 40 years old, characterizing early-onset parkinsonism, which differential diagnosis possibilities are varied. Atypical presentations include a pure akinetic-rigid syndrome, the initial manifestations occurring in the lower limbs, and pain as the most important or sole manifestation. These atypical features are unusual, but can be seen in clinical practice. We present a 37 years old woman with early-onset parkinsonism beginning with an akinetic-rigid syndrome in the lower limbs whose first symptom was left leg pain, which diagnosis was made after 4 years of onset and after 16 referrals to several experts in different fields. We discuss these atypical features and the diagnostic pitfalls in PD.A doença de Parkinson (DP caracteriza-se pela tríade de tremor, rigidez e bradicinesia. Em cerca de 80% dos casos inicia-se com tremor em membros superiores. Entretanto, certas atipias na apresentação desta entidade tornam seu diagnóstico ainda mais desafiador. A doença pode iniciar-se abaixo dos 40 anos de idade, caracterizando o parkinsonismo de início precoce. Apresentações atípicas incluem uma síndrome rígido-acinética pura, as manifestações inaugurais ocorrendo em membros inferiores, e uma síndrome dolorosa podendo ser um sintoma inicial proeminente ou o único sintoma. Estas atipias, apesar de infreqüentes, são encontradas na prática clínica diária. Discutimos estas características atípicas ao apresentarmos uma paciente de 37 anos com quadro rígido-acinético de instalação precoce, iniciado em membros inferiores, cujo sintoma predominante era dor, cujo diagnóstico somente foi feito após 4 anos do início e após 16 avalia

  12. Phylogenetic analysis and molecular signatures defining a monophyletic clade of heterocystous cyanobacteria and identifying its closest relatives. (United States)

    Howard-Azzeh, Mohammad; Shamseer, Larissa; Schellhorn, Herb E; Gupta, Radhey S


    Detailed phylogenetic and comparative genomic analyses are reported on 140 genome sequenced cyanobacteria with the main focus on the heterocyst-differentiating cyanobacteria. In a phylogenetic tree for cyanobacteria based upon concatenated sequences for 32 conserved proteins, the available cyanobacteria formed 8-9 strongly supported clades at the highest level, which may correspond to the higher taxonomic clades of this phylum. One of these clades contained all heterocystous cyanobacteria; within this clade, the members exhibiting either true (Nostocales) or false (Stigonematales) branching of filaments were intermixed indicating that the division of the heterocysts-forming cyanobacteria into these two groups is not supported by phylogenetic considerations. However, in both the protein tree as well as in the 16S rRNA gene tree, the akinete-forming heterocystous cyanobacteria formed a distinct clade. Within this clade, the members which differentiate into hormogonia or those which lack this ability were also separated into distinct groups. A novel molecular signature identified in this work that is uniquely shared by the akinete-forming heterocystous cyanobacteria provides further evidence that the members of this group are specifically related and they shared a common ancestor exclusive of the other cyanobacteria. Detailed comparative analyses on protein sequences from the genomes of heterocystous cyanobacteria reported here have also identified eight conserved signature indels (CSIs) in proteins involved in a broad range of functions, and three conserved signature proteins, that are either uniquely or mainly found in all heterocysts-forming cyanobacteria, but generally not found in other cyanobacteria. These molecular markers provide novel means for the identification of heterocystous cyanobacteria, and they provide evidence of their monophyletic origin. Additionally, this work has also identified seven CSIs in other proteins which in addition to the heterocystous

  13. Diagnosis and treatment of infratentorial rosette forming glioneuronal tumors%幕下菊形团形成型胶质神经元肿瘤的诊断和治疗

    Institute of Scientific and Technical Information of China (English)

    张忠; 王永志; 季玉陈; 陈宝师


    Objective To summarize clinical, imaging and pathological characteristics of the infratentorial rosette-forming glioneuronal tumor for improving the understanding of the disease. Methods Clinical data of 4 patients with surgically and pathologically verified rosette-forming glioneuronal tumor were analyzed retrospectively. The tumors were resected by micro-craniotomy via sub-occipital midline approach. Results Total removal was achieved in 3 patients and nearly total resection in 1. Mutism and ataxia occurred in 1 patient and diplopia in 1. All the patients were followed up for 6 to 24 months, no relapse was observed. The diplopia disappeared but there was no improvement in mutism. Conclusions The rosette-forming glioneuronal tumor is a rare benign tumor. Surgical resection is the most common treatment. Postoperative irradiation or chemotherapy is not recommended but further long-term follow-up is necessary.%目的:总结幕下菊形团形成型胶质神经元肿瘤的临床、影像、病理学特征,旨在提高对该病的认识。方法回顾性分析经手术及病理证实4例菊形团形成型胶质神经元肿瘤病人的临床资料,均在显微镜下行枕下后正中开颅切除肿瘤。结果全切3例,近全切1例。术后1例出现缄默伴共济失调,1例复视。随访6~24个月,均未见复发。复视症状消失,缄默症状无明显改善。结论菊形团形成型胶质神经元肿瘤是一种罕见的良性肿瘤。手术切除肿瘤是常见的治疗方式,术后一般不推荐放化疗,但应该长期随访。

  14. Eocene lizard from Germany reveals amphisbaenian origins. (United States)

    Müller, Johannes; Hipsley, Christy A; Head, Jason J; Kardjilov, Nikolay; Hilger, André; Wuttke, Michael; Reisz, Robert R


    Amphisbaenia is a speciose clade of fossorial lizards characterized by a snake-like body and a strongly reinforced skull adapted for head-first burrowing. The evolutionary origins of amphisbaenians are controversial, with molecular data uniting them with lacertids, a clade of Old World terrestrial lizards, whereas morphology supports a grouping with snakes and other limbless squamates. Reports of fossil stem amphisbaenians have been falsified, and no fossils have previously tested these competing phylogenetic hypotheses or shed light on ancestral amphisbaenian ecology. Here we report the discovery of a new lacertid-like lizard from the Eocene Messel locality of Germany that provides the first morphological evidence for lacertid-amphisbaenian monophyly on the basis of a reinforced, akinetic skull roof and braincase, supporting the view that body elongation and limblessness in amphisbaenians and snakes evolved independently. Morphometric analysis of body shape and ecology in squamates indicates that the postcranial anatomy of the new taxon is most consistent with opportunistically burrowing habits, which in combination with cranial reinforcement indicates that head-first burrowing evolved before body elongation and may have been a crucial first step in the evolution of amphisbaenian fossoriality.

  15. Determination of Kinetic Parameters for Methane Oxidation over Pt/γ-Al2O3 in a Fixed-Bed Reactor

    Directory of Open Access Journals (Sweden)

    Vita Wonoputri


    Full Text Available This paper describes akinetic study for the determination of the kinetic parameters of lean methane emission oxidation over Pt/γ-Al2O3 in a dedicated laboratory scale fixed bed reactor. A model ofthemechanistic reaction kinetic parameters has been developed. The reaction rate model was determined using therate-limiting step method, which was integrated and optimized to find the most suitable model and parameters. Based on this study, the Langmuir-Hinshelwood reaction rate model with the best correlationis the one where the rate-limiting step is thesurface reaction between methane and one adsorbed oxygen atom. The pre-exponential factor and activation energy were 9.19 x 105 and 92.04 kJ/mol, while the methane and oxygen adsorption entropy and enthalpy were –17.46 J/mol.K, –2739.36 J/mol,–16.34 J/mol.K, and –6157.09 J/mol, respectively.

  16. An exquisitely preserved troodontid theropod with new information on the palatal structure from the Upper Cretaceous of Mongolia (United States)

    Tsuihiji, Takanobu; Barsbold, Rinchen; Watabe, Mahito; Tsogtbaatar, Khishigjav; Chinzorig, Tsogtbaatar; Fujiyama, Yoshito; Suzuki, Shigeru


    Troodontidae is a clade of small-bodied theropod dinosaurs. A new troodontid, Gobivenator mongoliensis gen. et sp. nov., is described based on the most complete skeleton of a Late Cretaceous member of this clade presently known, from the Campanian Djadokhta Formation in the central Gobi Desert. G. mongoliensis is different from other troodontids in possessing a pointed anterior end of the fused parietal and a fossa on the surangular in front of the posterior surangular foramen. The skull was superbly preserved in the specimen and provides detailed information of the entire configuration of the palate in Troodontidae. Overall morphology of the palate in Gobivenator resembles those of dromaeosaurids and Archaeopteryx, showing an apparent trend of elongation of the pterygoid process of the palatine and reduction of the pterygopalatine suture toward the basal Avialae. The palatal configuration suggests that the skull of Gobivenator would have been akinetic but had already acquired prerequisites for later evolution of cranial kinesis in birds, such as the loss of the epipterygoid and reduction in contact areas among bones.

  17. Molecular phylogenetics of emydine turtles: taxonomic revision and the evolution of shell kinesis. (United States)

    Feldman, Chris R; Parham, James Ford


    The 10 extant species of emydine turtles represent an array of morphological and ecological forms recognizable and popular among scientists and hobbyists. Nevertheless, the phylogenetic affinities of most emydines remain contentious. Here, we examine the evolutionary relationships of emydine turtles using 2092 bp of DNA encoding the mitochondrial genes cyt b, ND4, and adjacent tRNAs. These data contain 339 parsimony informative characters that we use to erect hypotheses of relationships for the Emydinae. Both maximum parsimony and maximum likelihood methods yield a monophyletic Emydinae in which all but three nodes are well resolved. Emys orbicularis, Emydoidea blandingii, and Clemmys marmorata form a monophyletic clade, as do the species of Terrapene. Clemmys muhlenbergii and Clemmys insculpta form a third monophyletic group that may be sister to all other emydines. Clemmys guttata is problematic and probably related to Terrapene. Based on this phylogeny, and previous molecular work on the group, we suggest the following taxonomic revisions: (1) Clemmys should be restricted to a single species, C. guttata. (2) Calemys should be resurrected for C. muhlenbergii and C. insculpta. (3) Emys should be expanded to include three species: E. orbicularis, E. blandingii, and E. marmorata. Furthermore, our analyses show that neither kinetic-shelled nor akinetic-shelled emydines form monophyletic groups. Therefore, shell kinesis was either independently gained in Emys and Terrapene or secondarily lost in E. marmorata and C. guttata. Parsimony, paleontological evidence, and the multiple origins of shell kinesis in related turtle lineages (especially geoemydines) support the independent origin of plastral kinesis.

  18. The Nostoc punctiforme Genome

    Energy Technology Data Exchange (ETDEWEB)

    John C. Meeks


    Nostoc punctiforme is a filamentous cyanobacterium with extensive phenotypic characteristics and a relatively large genome, approaching 10 Mb. The phenotypic characteristics include a photoautotrophic, diazotrophic mode of growth, but N. punctiforme is also facultatively heterotrophic; its vegetative cells have multiple development alternatives, including terminal differentiation into nitrogen-fixing heterocysts and transient differentiation into spore-like akinetes or motile filaments called hormogonia; and N. punctiforme has broad symbiotic competence with fungi and terrestrial plants, including bryophytes, gymnosperms and an angiosperm. The shotgun-sequencing phase of the N. punctiforme strain ATCC 29133 genome has been completed by the Joint Genome Institute. Annotation of an 8.9 Mb database yielded 7432 open reading frames, 45% of which encode proteins with known or probable known function and 29% of which are unique to N. punctiforme. Comparative analysis of the sequence indicates a genome that is highly plastic and in a state of flux, with numerous insertion sequences and multilocus repeats, as well as genes encoding transposases and DNA modification enzymes. The sequence also reveals the presence of genes encoding putative proteins that collectively define almost all characteristics of cyanobacteria as a group. N. punctiforme has an extensive potential to sense and respond to environmental signals as reflected by the presence of more than 400 genes encoding sensor protein kinases, response regulators and other transcriptional factors. The signal transduction systems and any of the large number of unique genes may play essential roles in the cell differentiation and symbiotic interaction properties of N. punctiforme.

  19. Medication Responsiveness of Motor Symptoms in a Population-Based Study of Parkinson Disease

    Directory of Open Access Journals (Sweden)

    Yvette M. Bordelon


    Full Text Available We assessed degree of Parkinson disease motor symptom improvement with medication among subjects enrolled in an ongoing, population-based study in Central California. The motor section of the unified Parkinson disease rating scale (UPDRS was performed on subjects in both OFF and ON medication states, and difference between these scores was used as an indicator of symptomatic benefit. Higher OFF minus ON scores correlated with more severe baseline symptoms. There was equivalent improvement on the motor UPDRS scale for subjects divided according to medication classes used: levodopa alone 7.3 points, levodopa plus other medications 8.5 points, and dopamine agonists but not levodopa 6.1 points. In addition, there was no difference in the magnitude of improvement when subjects were divided according to Parkinson disease subtype, defined as tremor dominant, akinetic-rigid, or mixed. In this community-based sample, these values are within the range of a clinically important difference as defined by previous studies.

  20. Assessment of the role of sutures in a lizard skull: a computer modelling study. (United States)

    Moazen, Mehran; Curtis, Neil; O'Higgins, Paul; Jones, Marc E H; Evans, Susan E; Fagan, Michael J


    Sutures form an integral part of the functioning skull, but their role has long been debated among vertebrate morphologists and palaeontologists. Furthermore, the relationship between typical skull sutures, and those involved in cranial kinesis, is poorly understood. In a series of computational modelling studies, complex loading conditions obtained through multibody dynamics analysis were imposed on a finite element model of the skull of Uromastyx hardwickii, an akinetic herbivorous lizard. A finite element analysis (FEA) of a skull with no sutures revealed higher patterns of strain in regions where cranial sutures are located in the skull. From these findings, FEAs were performed on skulls with sutures (individual and groups of sutures) to investigate their role and function more thoroughly. Our results showed that individual sutures relieved strain locally, but only at the expense of elevated strain in other regions of the skull. These findings provide an insight into the behaviour of sutures and show how they are adapted to work together to distribute strain around the skull. Premature fusion of one suture could therefore lead to increased abnormal loading on other regions of the skull causing irregular bone growth and deformities. This detailed investigation also revealed that the frontal-parietal suture of the Uromastyx skull played a substantial role in relieving strain compared with the other sutures. This raises questions about the original role of mesokinesis in squamate evolution.

  1. Off-Pump Coronary Bypass Grafting Causing Stunned Myocardium

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    Feridoun Sabzi


    Full Text Available The term “stunned myocardium” refers to abnormalities in the myocardial function following reperfusion and is common in on-pump coronary artery bypass grafting (CABG and is exceedingly rare in off- pump CABG. A 53-year-old man presented with unstable angina due to the severe stenosis of the left anterior descending coronary artery (LAD and the obtuse marginal. Laboratory findings and Chest X-ray revealed nothing abnormal. The intraoperative course was uneventful. The patient left the operating room without any inotropic support. Six hours later, however, he developed low cardiac output .At exploration, cardiac tamponade was excluded and flowmetry showed that the graft had adequate function. Cardiac enzymes were normal. High-dose adrenalin and Dobutamine were administrated and an intra-aortic balloon pump was used. After hemodynamic stabilization, the patient left the Intensive Care Unit without an intra-aortic balloon pump and inotropic support. On the fifth postoperative day, coronary angiography showed patent grafts and correct anastomotic sites. On the seventh postoperative day, the akinetic lateral wall of the left ventricle changed to dyskinesia. Finally after hospital discharge on the thirtieth postoperative day, an echocardiogram showed normal left ventricular function without regional wall motion abnormalities

  2. Repeated renal infarction in native and transplanted kidneys due to left ventricular thrombus formation caused by antiphospholipid antibody syndrome

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    Scully P


    Full Text Available Paul Scully,1 Daniel C Leckstroem,1 Andrew McGrath,2 John Chambers,3 David J Goldsmith11Nephrology Department, 2Radiology Department, 3Cardiology Department, King's Health Partners, Academic Health Sciences Centre, London, United KingdomAbstract: Antiphospholipid syndrome can be a feature of several underlying conditions, such as lupus, but it can also occur idiopathically. Diagnosis usually comes after investigation of recurrent venous or arterial thromboses, emboli, or hypertension/proteinuria where the kidney is involved and is usually confirmed by laboratory testing. We describe a case of a man with a myocardial infarction who developed mural thrombus in an akinetic left ventricular segment but then who recurrently embolized first to one of his native kidneys and then later to a transplanted kidney. Although the clinical behavior was typical of antiphospholipid syndrome, it took numerous laboratory assays over many years until finally the problem was confirmed and life-long warfarin therapy instituted.Keywords: antiphospholipid therapy, emboli, infarction, kidney, kidney transplant

  3. Movement disorders and the osmotic demyelination syndrome. (United States)

    de Souza, Aaron


    With the advent of MRI, osmotic demyelination syndromes (ODS) are increasingly recognised to affect varied sites in the brain in addition to the classical central pontine lesion. Striatal involvement is seen in a large proportion of cases and results in a wide variety of movement disorders. Movement disorders and cognitive problems resulting from ODS affecting the basal ganglia may occur early in the course of the illness, or may present as delayed manifestations after the patient survives the acute phase. Such delayed symptoms may evolve over time, and may even progress despite treatment. Improved survival of patients in the last few decades due to better intensive care has led to an increase in the incidence of such delayed manifestations of ODS. While the outcome of ODS is not as dismal as hitherto believed - with the acute akinetic-rigid syndrome associated with striatal myelinolysis often responding to dopaminergic therapy - the delayed symptoms often prove refractory to medical therapy. This article presents a review of the epidemiology, pathophysiology, clinical features, imaging, and therapy of movement disorders associated with involvement of the basal ganglia in ODS. A comprehensive review of 54 previously published cases of movement disorders due to ODS, and a video recording depicting the spectrum of delayed movement disorders seen after recovery from ODS are also presented.

  4. Stress-induced cardiomyopathy (Takotsubo – broken heart and mind?

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    Redfors B


    Full Text Available Björn Redfors, Yangzhen Shao, Elmir Omerovic Department of Molecular and Clinical Medicine, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden Abstract: Stress-induced cardiomyopathy (SIC, also known as Takotsubo cardiomyopathy, is characterized by severe but potentially reversible regional left ventricular wall motion abnormalities, ie, akinesia, in the absence of explanatory angiographic evidence of a coronary occlusion. The typical pattern is that of an akinetic apex with preserved contractions in the base, but other variants are also common, including basal or midmyocardial akinesia with preserved apical function. The pathophysiology of SIC remains largely unknown but catecholamines are believed to play a pivotal role. The diverse array of triggering events that have been linked to SIC are arbitrarily categorized as either emotional or somatic stressors. These categories can be considered as different elements of a continuous spectrum, linked through the interface of neurology and psychiatry. This paper reviews our current knowledge of SIC, with focus on the intimate relationship between the brain and the heart. Keywords: stress-induced cardiomyopathy, takotsubo cardiomyopathy, catecholamine, cerebral injury, emotional stress, somatic stress

  5. Biventricular Failure due to Stress Cardiomyopathy after Pericardiectomy for Constrictive Pericarditis

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    Elliott M. Groves


    Full Text Available Importance. Constrictive pericarditis is a rare clinical entity that frequently necessitates surgical intervention. Here we present a case of biventricular failure due to stress cardiomyopathy after pericardiectomy. This is an extremely rare complication that is not well described and does not have a definitive mechanism. Observations. A 40-year-old Ecuadorian woman who was found to have constrictive pericarditis due to Mycobacterium tuberculosis infection was referred to our institution. The presence of constrictive pericarditis was confirmed by echocardiography, computed tomography, magnetic resonance imaging, and cardiac catheterization. Following pericardiectomy, the patient developed biventricular failure consistent with stress cardiomyopathy (Takotsubo cardiomyopathy, based on the echocardiographic assessment of the ventricles, which demonstrated an akinetic apex and hyperactive base in both ventricles, the absence of significant epicardial coronary atherosclerosis, and prompt normalization of the cardiac function after intensive medical therapy. Conclusions and Relevance. Biventricular failure in the form of stress cardiomyopathy after pericardiectomy in the manner presented here has not been previously described in the literature. While postulations as to the cause of single ventricle dysfunction have been described, the exact mechanism is unclear and current theories do not explain the clinical features in this case of stress cardiomyopathy after pericardiectomy.

  6. Systematic mutation analysis of KIAA0767 and KIAA1646 in chromosome 22q-linked periodic catatonia

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    Gawlik Micha


    Full Text Available Abstract Background Periodic catatonia is a familial subtype of schizophrenia characterized by hyperkinetic and akinetic episodes, followed by a catatonic residual syndrome. The phenotype has been evaluated in two independent genome-wide linkage scans with evidence for a major locus on chromosome 15q15, and a second independent locus on chromosome 22qtel. Methods In the positional and brain-expressed candidate genes KIAA0767 and KIAA1646, we searched for variants in the complete exons and adjacent splice-junctions as well as in parts of the 5'- and 3'-untranslated regions by means of a systematic mutation screening in individuals from chromosome 22q-linked pedigrees. Results The mutation scan revealed 24 single nucleotide polymorphisms, among them two rare codon variants (KIAA0767: S159I; KIAA1646: V338G. However, both were neither found segregating with the disease in the respective pedigree nor found at a significant frequency in a case-control association sample. Conclusion Starting from linkage signals at chromosome22qtel in periodic catatonia, we screened two positional brain-expressed candidate genes for genetic variation. Our study excludes genetic variations in the coding and putative promoter regions of KIAA0767 and KIAA1646 as causative factors for periodic catatonia.

  7. Righting elicited by novel or familiar auditory or vestibular stimulation in the haloperidol-treated rat: rat posturography as a model to study anticipatory motor control. (United States)

    Clark, Callie A M; Sacrey, Lori-Ann R; Whishaw, Ian Q


    External cues, including familiar music, can release Parkinson's disease patients from catalepsy but the neural basis of the effect is not well understood. In the present study, posturography, the study of posture and its allied reflexes, was used to develop an animal model that could be used to investigate the underlying neural mechanisms of this sound-induced behavioral activation. In the rat, akinetic catalepsy induced by a dopamine D2 receptor antagonist (haloperidol 5mg/kg) can model human catalepsy. Using this model, two experiments examined whether novel versus familiar sound stimuli could interrupt haloperidol-induced catalepsy in the rat. Rats were placed on a variably inclined grid and novel or familiar auditory cues (single key jingle or multiple key jingles) were presented. The dependent variable was movement by the rats to regain equilibrium as assessed with a movement notation score. The sound cues enhanced movements used to regain postural stability and familiar sound stimuli were more effective than unfamiliar sound stimuli. The results are discussed in relation to the idea that nonlemniscal and lemniscal auditory pathways differentially contribute to behavioral activation versus tonotopic processing of sound.

  8. Palaeobiology of Mesoproterozoic Salkhan Limestone, Semri Group, Rohtas, Bihar, India: Systematics and significance

    Indian Academy of Sciences (India)

    Mukund Sharma


    Mesoproterozoic (∼1600 Ma old) Salkhan Limestone (Semri Group)of the Vindhyan Supergroup, exposed in Rohtas district of Bihar,India,preserves an abundant and varied ancient microbial assemblage.These microfossils are recorded in three distinctly occurring cherts viz.,bedded chert, stromatolitic chert and cherty stromatolites.27 morphoforms belonging to 14 genera and 21 species have been recognized.Six unnamed forms are also described. The microbial assemblage,almost exclusively composed of the remnants of cyanobacteria,is dominated by entophysalidacean members and short trichomes and can be termed as ‘typical Meso-proterozoic microbiotas ’.The assemblage includes characteristic mat-forming scytonematacean and entophysalidacean cyanobacteria. Eoentophysalis is the dominant organism in the assemblage. Ellipsoidal akinetes of nostocalean cyanobacteria (Archaeollipsoides) and spherical unicells also occur;both are distinct from mat forming assemblage,allochthonous and possibly planktic.Co-occurrence of the microbiotas and precipitates is related to the depositional environment of the Mesoproterozoic tidal flats with high carbonate saturation.

  9. A comparative study on neurochemistry of cerebrospinal fluid in advanced Parkinson's disease. (United States)

    Liu, H; Iacono, R P; Schoonenberg, T; Kuniyoshi, S; Buchholz, J


    This study addresses two issues: (1) the comparative neurochemistry of classic tremor type of Parkinson's disease or PD-A and akinetic type of Parkinson's disease or PD-B; and (2) the neurochemistry of levodopa failure syndrome (LDFS). Cerebrospinal fluid from the lateral ventricle was collected from 50 patients with idiopathic Parkinson's disease of PD-A and PD-B. Levels of monoamine neurotransmitters and metabolites were determined using high performance liquid chromatography. We have found that (1) 5-hydroxylindoleacetic acid (5-HIAA) level is significantly lower in PD-B than in PD-A; (2) 5-HIAA level is inversely associated with score of part one of United Parkinson's Disease Rating Score (UPDRS); (3) 5-HIAA level is inversely associated with score of part four of UPDRS; (4) 3-O-methyldopa (3-OMD) level is positively associated with levodopa failure syndrome (LDFS) assessed by part four of UPDRS and inversely associates with 5-HIAA. From these data, it can be inferred that serotonergic activity is decreased in PD-B to a greater extent than in PD-A and that decreased serotonergic activity plays a role in LDFS.

  10. Brain Perfusion in Corticobasal Syndrome with Progressive Aphasia

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    Yoshitake Abe


    Full Text Available Background: Brain perfusion may differ between patients with corticobasal syndrome (CBS with and without aphasia. Methods: Twenty-six (9 males and 17 females; mean age 76 ± 5.3 years patients with CBS were enrolled in the study. Brain MRI and single-photon emission computed tomography were performed in all subjects. Language was evaluated using the Standard Language Test of Aphasia. The patients were divided into two subgroups according to the presence or absence of progressive aphasia. Differences in the regional cerebral blood flow (rCBF between the two groups were detected based on voxel-by-voxel group analysis using Statistical Parametric Mapping 8. Results: All patients exhibited asymmetric motor symptoms and signs, including limb apraxia, bradykinesia, and akinetic rigidity. Of 26 patients, 9 had a clinically obvious language disturbance, characterized as nonfluent aphasia. Almost all CBS patients with aphasia exhibited cortical atrophy predominantly in the left frontal and temporal lobes with widening of the Sylvian fissure on MRI. The rCBF in the left middle frontal gyrus differed significantly between CBS patients with and without aphasia. Conclusion: CBS patients with aphasia exhibit motor symptoms predominantly on the right side and cortical atrophy mainly in the left perisylvian cortices. In particular, left frontal dysfunction might be related to nonfluent aphasia in CBS.


    Institute of Scientific and Technical Information of China (English)

    吴忠兴; 曾波; 李仁辉; 宋立荣


    our laboratory had shown that three types of Aphanizomenon, A. Flos-aquae, A. Gracile and A. Issatschenkoi, were the most common strains in Chinese freshwater bodies. Due to the limitation of samples and knowledge, however, the physiological characters were not compared in the three-type strains so far. Therefore, in order to further explore their knowledge and investigate the bloom-forming mechanism in Aphanizomenon, in the present study, their morphological and physiological characteristics, including the ratio of length and width in vegetative cell, heterocyst and akinete, growth rate, pigment composition, photosynthetic O2 evolution, and the electron transport rate (ETR), were compared in A. Flos-aquae, A. Gracile and A. Issatschenkoi. The results indicated that some morphological differences were found in the frequency distributions for length/width ratios of vegetative cells, heterocyst, and akinetes. Among them, the most sig nificant difference was found in the akinetes, suggesting that the length/ width ratios of akinetes could be regarded as a taxonomic unit in the genus Aphanizomenon. Moreover, physiological differences were also showed in three types of Aphanizomenon. Compared with the strains of A. Flos-aquae and A. Issatschenkoi, the strains of A. Gracile displayed higher PC contents. However, the strains of A. Gracile showed significantly lower chlorophyll a, carotenoid contents, maximum photosynthesis (Pm), apparent photosynthetic efficiency (a), and maximal electron transport rates (ETRmax) in comparison with the other strains of Aphanizomenon. It suggested that the strain of A. Gracile showed lower competitive abilities in photosynthesis when compared with the strains of A. Flos-aquae and A. Issatschenkoi. In addition, similar physiological features, such as the value of Pm and ETRmax, pigment contents and specific growth rate (μ), were found in A. Flos-aquae and A. Issatschenkoi. These data suggested that the three morphological strains of

  12. Juvenile Huntington's disease confirmed by genetic examination in twins Doença de Huntington juvenil confirmada por exame genético em gêmeas

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    Full Text Available Early-onset Huntington's disease (HD occurs in approximately 10% of HD's cases. We report juvenile HD in phenotypically identical twins, evaluated by history, clinical and neurologic examination, mini-mental state examination, blood laboratory exams, cerebrospinal fluid examination, skull computed tomography, and genetic examination for HD. Patients had the akinetic-rigid variety (Westphal variant of the disease and paternal inheritance. The laboratory workup confirmed the clinical diagnosis of HD, which adds this report to the rare cases of HD in twins reported in the literature.Doença de Huntington (DH de início precoce ocorre em aproximadamente 10% dos casos de DH. Relatamos DH juvenil em gêmeas fenotipicamente idênticas, avaliadas por história, exames clínico e neurológico, mini-exame do estado mental, exames de sangue, exame do líquido cefalorraquidiano, tomografia computadorizada de crânio e exame genético para DH. As pacientes apresentavam a variedade rígido-acinética (variante de Westphal da doença e herança paterna. A avaliação laboratorial confirmou o diagnóstico clínico de DH, acrescentando-se este relato aos raros casos de DH em gêmeos relatados na literatura.

  13. Postharvest treatment of fresh fruit from California with methyl bromide for control of light brown apple moth (Lepidoptera: Tortricidae). (United States)

    Walse, Spencer S; Myers, Scott W; Liu, Yong-Biao; Bellamy, David E; Obenland, David; Simmons, Greg S; Tebbets, Steve


    Methyl bromide (MB) chamber fumigations were evaluated for postharvest control of light brown apple moth, Epiphyas postvittana (Walker) (Lepidoptera: Tortricidae), in fresh fruit destined for export from California. To simulate external feeding, larvae were contained in gas-permeable cages and distributed throughout loads of peaches, plums, nectarines (all Prunus spp.), apples (Malus spp.), raspberries (Rubus spp.), or grapes (Vitis spp.). Varying the applied MB dose and the differential sorption of MB by the loads resulted in a range of exposures, expressed as concentration x time cross products (CTs) that were verified by gas-chromatographic quantification of MB in chamber headspace over the course of each fumigation. CTs > or = 60 and > or = 72 mg liter(-1) h at 10.0 +/- 0.5 and 15.6 +/- 0.5 degrees C (x +/- s, average +/- SD), respectively, yielded complete mortality of approximately 6,200 larvae at each temperature. These confirmatory fumigations corroborate E. postvittana mortality data for the first time in relation to measured MB exposures and collectively comprise the largest number of larval specimens tested to date. In addition, akinetic model of MB sorption was developed for the quarantine fumigation of fresh fruit based on the measurement of exposures and how they varied across the fumigation trials. The model describes how to manipulate the applied MB dose, the load factor, and the load geometry for different types of packaged fresh fruit so that the resultant exposure is adequate for insect control.

  14. Variations of Growth and Toxin Yield in Cylindrospermopsis raciborskii under Different Phosphorus Concentrations

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    Yiming Yang


    Full Text Available The bloom-forming cyanobacteria, Cylindrospermopsis raciborskii, is a producer of the cytotoxic cylindrospermopsin (CYN. In this study, the growth, toxin yield, and expression of CYN biosynthesis genes of C. raciborskii were examined under varying phosphorus (P concentrations. The results show the cell number at 0.00 and 0.01 mg·L−1 P was significantly lower than that at higher P concentrations (≥0.5 mg·L−1. The chlorophyll a content, filament length, heterocyst, and akinete numbers at P ≤ 0.05 mg·L−1 were also significantly reduced. The intracellular and extracellular CYN concentrations and the extracellular proportions increased during the culture period, and larger values were observed at higher P concentrations. Total CYN content reached 45.34–63.83 fg·cell−1 and extracellular CYN proportion reached 11.49%–20.44% at the stationary growth phase. A significantly positive correlation was observed between CYN production and cell growth rate. Three cyr genes were expressed constantly even at P-deficient conditions. The transcription of cyr genes at P-replete conditions or after P supplementation increased from 1.18-fold to 8.33-fold. In conclusion, C. raciborskii may rapidly reorganize metabolic processes as an adaptive response to environmental P fluctuations. CYN production and cyr gene expression were constitutive metabolic processes in toxic C. raciborskii.

  15. Responses of a rice-field cyanobacterium Anabaena siamensis TISTR-8012 upon exposure to PAR and UV radiation. (United States)

    Rastogi, Rajesh P; Incharoensakdi, Aran; Madamwar, Datta


    The effects of PAR and UV radiation and subsequent responses of certain antioxidant enzymatic and non-enzymatic defense systems were studied in a rice field cyanobacterium Anabaena siamensis TISTR 8012. UV radiation resulted in a decline in growth accompanied by a decrease in chlorophyll a and photosynthetic efficiency. Exposure of cells to UV radiation significantly affected the differentiation of vegetative cells into heterocysts or akinetes. UV-B radiation caused the fragmentation of the cyanobacterial filaments conceivably due to the observed oxidative stress. A significant increase of reactive oxygen species in vivo and DNA strand breaks were observed in UV-B exposed cells followed by those under UV-A and PAR radiation, respectively. The UV-induced oxidative damage was alleviated due to an induction of antioxidant enzymatic/non-enzymatic defense systems. In response to UV irradiation, the studied cyanobacterium exhibited a significant increase in antioxidative enzyme activities of superoxide dismutase, catalase and peroxidase. Moreover, the cyanobacterium also synthesized some UV-absorbing/screening substances. HPLC coupled with a PDA detector revealed the presence of three compounds with UV-absorption maxima at 326, 331 and 345 nm. The induction of the biosynthesis of these UV-absorbing compounds was found under both PAR and UV radiation, thus suggesting their possible function as an active photoprotectant.

  16. Hindcasting cyanobacterial communities in Lake Okaro with germination experiments and genetic analyses. (United States)

    Wood, Susanna A; Jentzsch, Katrin; Rueckert, Andreas; Hamilton, David P; Cary, S Craig


    Cyanobacterial blooms are becoming increasingly prevalent worldwide. Sparse historic phytoplankton records often result in uncertainty as to whether bloom-forming species have always been present and are proliferating in response to eutrophication or climate change, or if there has been a succession of new arrivals through recent history. This study evaluated the relative efficacies of germination experiments and automated rRNA intergenic spacer analysis (ARISA) assays in identifying cyanobacteria in a sediment core and thus reconstructing the historical composition of cyanobacterial communities. A core (360 mm in depth) was taken in the central, undisturbed basin of Lake Okaro, New Zealand, a lake with a rapid advance of eutrophication and increasing cyanobacteria populations. The core incorporated a tephra from an 1886 volcanic eruption that served to delineate recent sediment deposition. ARISA and germination experiments successfully detected akinete-forming nostocaleans in sediment dating 120 bp and showed little change in Nostocales species structure over this time scale. Species that had not previously been documented in the lake were identified including Aphanizomenon issatschenkoi, a potent anatoxin-a producer. The historic composition of Chrococcales and Oscillatoriales was more difficult to reconstruct, potentially due to the relatively rapid degradation of vegetative cells within sediment.

  17. Longitudinal strain is a marker of microvascular obstruction and infarct size in patients with acute ST-segment elevation myocardial infarction.

    Directory of Open Access Journals (Sweden)

    Loïc Bière

    Full Text Available OBJECTIVES: We assessed the value of speckle tracking imaging performed early after a first ST-segment elevation myocardial infarction (STEMI in order to predict infarct size and functional recovery at 3-month follow-up. METHODS: 44 patients with STEMI who underwent revascularization within 12 h of symptom onset were prospectively enrolled. Echocardiography was performed 3.9 ± 1.2 days after myocardial reperfusion, assessing circumferential (CGS, radial (RGS, and longitudinal global (GLS strains. Late gadolinium-enhanced cardiac magnetic imaging (CMR, for assessing cardiac function, infarct size, and microvascular obstruction (MVO, was conducted 5.6 ± 2.5 days and 99.4 ± 4.6 days after myocardial reperfusion. RESULTS: GLS was evaluable in 97% of the patients, while CGS and RGS could be assessed in 85%. Infarct size significantly correlated with GLS (R = 0.601, p-6.0% within the infarcted area exhibited 96% specificity and 61% sensitivity for predicting the persistence of akinesia (≥ 3 segments at 3-month follow-up. CONCLUSIONS: Speckle-tracking strain imaging performed early after a STEMI is easy-to-use as a marker for persistent akinetic territories at 3 months. In addition, GLS correlated significantly with MVO and final infarct size, both parameters being relevant post-MI prognostic factors, usually obtained via CMR.

  18. Dopamine imbalance in Huntington's Disease: a mechanism for the lack of behavioral flexibility

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    Jane Y Chen


    Full Text Available Dopamine (DA plays an essential role in the control of coordinated movements. Alterations in DA balance in the striatum lead to pathological conditions such as Parkinson’s and Huntington’s diseases (HD. HD is a progressive, invariably fatal neurodegenerative disease caused by a genetic mutation producing an expansion of glutamine repeats and is characterized by abnormal dance-like movements (chorea. The principal pathology is the loss of striatal and cortical projection neurons. Changes in brain DA content and receptor number contribute to abnormal movements and cognitive deficits in HD. In particular, during the early hyperkinetic stage of HD, DA levels are increased whereas expression of DA receptors is reduced. In contrast, in the late akinetic stage, DA levels are significantly decreased and resemble those of a Parkinsonian state. Time-dependent changes in DA transmission parallel biphasic changes in glutamate synaptic transmission and may enhance alterations in glutamate receptor-mediated synaptic activity. In this review, we focus on neuronal electrophysiological mechanisms that may lead to some of the motor and cognitive symptoms of HD and how they relate to dysfunction in DA neurotransmission. Based on clinical and experimental findings, we propose that some of the behavioral alterations in HD, including reduced behavioral flexibility, may be caused by altered DA modulatory function. Thus, restoring DA balance alone or in conjunction with glutamate receptor antagonists could be a viable therapeutic approach.

  19. [A case of malignant syndrome with leukoencephalopathy due to cocaine abuse]. (United States)

    Yamazaki, K; Katayama, S; Iwai, T; Hirata, K


    We report a rare case of malignant syndrome with leukoencephalopathy due to cocaine abuse. The patient was a 22-year-old man. After nasally ingesting 4.2 g of cocaine, he developed coma, high grade fever of 39 degrees C, excessive sweating, muscular rigidity, and elevation of CK to 61,240 mU/ml. Although these symptoms improved in about 2 weeks, diffuse low density area of the cerebral white matter was observed in CT scan, and T2-weighted MRI revealed a high signal intensity area. Since disorientation and abnormal behavior appeared on the 24th hospital day after a lucid interval, sulpiride and haloperidol were administered. The patient again had fever, akinetic mutisn, severe muscular rigidity and excessive sweating. This patient has malignant syndrome associated with a wide-ranging lesion of the cerebral white matter as a result of cocaine abuse. And then antipsychotic drugs were administered for mental symptoms that recurred after a lucid interval, and second bouts of neuroleptic malignant syndrome occurred. There have been only a few reports on malignant syndrome due to cocaine, and there have been no reports on leukoencephalopathy due to cocaine or malignant syndrome.

  20. Evaluation of myocarditis using Tl-201 myocardial scintigraphy with Bull's eye functional map

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    Satoh, Keiko; Utsumi, Jinji; Satoh, Hiroyuki (Juntendo Univ., Tokyo (Japan). School of Medicine) (and others)


    In order to evaluate myocardial damage in a patient with myocarditis, rest thallium-201 myocardial single photon emission computed tomography (SPECT) was performed in 15 patients with myocarditis. For qualitative and semiquantitative analysis, Bull's eye functional maps were made up with SPECT images. In the functional map, the abnormal area, where Tl uptake is less than mean-2SD of the Tl uptake of normal subjects, is generally distributed in the myocarditis group. But focal and sequential abnormal areas were recognized more often in the clinically severe cases. Abnormal area tended to be observed commonly at the antero-septal wall, but it was uncommon at the lateral wall. Extent score, i.e. degree of extension of abnormal area, and severity score, i. e. degree of abnormality, were in good negative correlation with left ventricular ejection fraction (r=0.6, r=0.7). Furthermore, existence of abnormal area was in good correlation with the left ventricular regional wall motion. Abnormal area existed 100% in the akinetic region , 71% in the region of severe hypokinesis, and 27% in the region of hypokinesis. Abnormal area occupied 30% of the normokinetic region in the myocarditis group, which was a higher rate than in the normal control group (p<0.05). It was suggested that latent myocardial damage existed in the normokinetic myocardium with myocarditis. Thus, rest Tl-201 SPECT with Bull's eye map is useful for clinical diagnosis in patients with myocarditis. (author).

  1. [The relation of electric myocardial instability to the intensity of coronary disease--correlation of the data of programmed electrostimulation of the ventricles, selective coronarography and contrast ventriculography]. (United States)

    Kaĭk, Iu L; Vaĭnu, M K; Makhotina, V N; Mezikepp, A Kh


    Altogether 104 persons with coronary disease were examined in order to determine the correlation between the presence of electric myocardial instability (EMI) identified with the aid of programmed electrostimulation of the ventricles (PESV) and the gravity of injuries to the coronary vessels and to the contractile myocardium of the left ventricle (LV). In accordance with the number of the stimulation-induced recurrent contractions (RC), the patients were distributed into 4 groups. The patients of the second group (4 to 9 RC), of the third group (with unstable tachycardia) and of the fourth group (with stable tachycardia) were considered as having EMI. A marked relationship was discovered between EMI identified with the aid of stimulation and the condition of the contractile myocardium of the LV according to the ventriculography readings (the frequency of recording akinetic and dyskinetic myocardial segments, the magnitude of the end-diastolic pressure). Coronary circulation failure (the degree of stenotic injury to the coronary arteries, the number of impaired vessels) did not determine EMI according to the PESV data.

  2. Accuracy of Dobutamine Stress Echocardiography in Detecting Recovery of Contractile Reserve after Revascularization of Ischemic Myocardium

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    Abas Ali karimi


    Full Text Available Background: This study was designed to investigate the accuracy of dobutamine stress echocardiography (DSE in detecting the post-revascularization recovery rate of contractile reserve (CR in ischemic myocardium. Methods: A total of 112 segments from seven patients with low ejection fraction (<35% and coronary artery disease were evaluated with DSE one week before and 12 weeks after coronary artery bypass graft surgery (CABG. Sensitivity, specificity, and positive and negative predictive values of DSE for detecting the recovery rate of CR were calculated based upon their standard definition and were presented with 95% confidence intervals (CI. Results: The mean baseline left ventricular ejection fraction was 31±4%, which reached 35±7% after CABG unremarkably. The recovery rates of resting function and CR were 18.2% and 50% for hypokinetic and 15.6% and 24.1 for akinetic segments respectively. Specificity, sensitivity, and positive and negative predictive values of DSE for detecting the recovery of CR were 83% (CI=69-97, 89% (CI=83-96, 94% (CI = 88-99, and 73 % (CI = 55-88, respectively. Conclusion: Despite acceptable sensitivity, specificity, and positive predictive value, DSE has a relatively lower negative predictive value for detecting the recovery of CR in ischemic myocardium and, consequently, the full extent of myocardial viability. Further sensitive techniques may, therefore, be needed to provide complementary information regarding long-term functional outcome.

  3. Exposure of phototrophs to 548 days in low Earth orbit: microbial selection pressures in outer space and on early earth. (United States)

    Cockell, Charles S; Rettberg, Petra; Rabbow, Elke; Olsson-Francis, Karen


    An epilithic microbial community was launched into low Earth orbit, and exposed to conditions in outer space for 548 days on the European Space Agency EXPOSE-E facility outside the International Space Station. The natural phototroph biofilm was augmented with akinetes of Anabaena cylindrica and vegetative cells of Nostoc commune and Chroococcidiopsis. In space-exposed dark controls, two algae (Chlorella and Rosenvingiella spp.), a cyanobacterium (Gloeocapsa sp.) and two bacteria associated with the natural community survived. Of the augmented organisms, cells of A. cylindrica and Chroococcidiopsis survived, but no cells of N. commune. Only cells of Chroococcidiopsis were cultured from samples exposed to the unattenuated extraterrestrial ultraviolet (UV) spectrum (>110 nm or 200 nm). Raman spectroscopy and bright-field microscopy showed that under these conditions the surface cells were bleached and their carotenoids were destroyed, although cell morphology was preserved. These experiments demonstrate that outer space can act as a selection pressure on the composition of microbial communities. The results obtained from samples exposed to >200 nm UV (simulating the putative worst-case UV exposure on the early Earth) demonstrate the potential for epilithic colonization of land masses during that time, but that UV radiation on anoxic planets can act as a strong selection pressure on surface-dwelling organisms. Finally, these experiments have yielded new phototrophic organisms of potential use in biomass and oxygen production in space exploration.

  4. The 7q11.23 Microduplication Syndrome: A Clinical Report with Review of Literature. (United States)

    Abbas, Elham; Cox, Devin M; Smith, Teri; Butler, Merlin G


    We report a 14-year-old adolescent girl with selective mutism (SM) and a 7q11.23 microduplication detected by chromosomal microarray (CMA) analysis and reviewed the literature from 18 published clinical reports. Our patient had specific phobias, SM, extreme anxiety, obesity, cutis marmorata, and a round appearing face with a short neck and over folded ears. We reviewed the published clinical, cognitive, behavioral, and cytogenetic findings grouped by speech and language delay, growth and development, craniofacial, clinical, and behavior and cognitive features due to the 7q11.23 microduplication. This microduplication syndrome is characterized by speech delay (91%), social anxiety (42%), attention deficit hyperactivity disorder (ADHD, 37%), autism spectrum disorder (29%), and separation anxiety (13%). Other findings include abnormal brain imaging (80%), congenital heart and vascular defects (54%), and mild intellectual disability (38%). We then compared the phenotype with Williams-Beuren syndrome (WBS) which is due to a deletion of the same chromosome region. Both syndromes have abnormal brain imaging, hypotonia, delayed motor development, joint laxity, mild intellectual disability, ADHD, autism, and poor visuospatial skills but opposite or dissimilar findings regarding speech and behavioral patterns, cardiovascular problems, and social interaction. Those with WBS are prone to have hyperverbal speech, lack of stranger anxiety, and supravalvular aortic stenosis while those with the 7q11.23 microduplication have speech delay, SM, social anxiety, and are prone to aortic dilatation.

  5. Neuroleptic malignant syndrome or a statin drug reaction? A case report. (United States)

    Cooper, Joyce M; Jones, Alison L


    A 60-year-old woman with a long psychiatric history presented with delirium and mutism. She was febrile, with marked limb rigidity and elevated creatinine kinase (CK) level. Current medications included pericyazine. Current or recent use of dopamine-blocking agents, such as pericyazine, together with a disturbance in conscious state, autonomic dysfunction, and an elevated CK level may be suggestive of neuroleptic malignant syndrome (NMS). The diagnosis was confirmed as NMS, and she was successfully treated with bromocriptine. Eight years later, she represents with symptoms suggesting recurrence of NMS including elevated CK level and myalgia, however, without limb rigidity. Current medications include quetiapine, lithium, simvastatin, and a recent course of clarithromycin. Macrolide antibiotics such as clarithromycin inhibit the metabolic pathway of statins via the cytochrome CYP450 3A4 hepatic enzyme system and may result in elevated CK level, myopathy, or rhabdomyolysis producing symptoms that may be confused with NMS. Simvastatin was ceased with rapid decrease in CK level and resolution of symptoms. This case highlights the importance of considering other diagnoses in any patient presenting with a disturbance in conscious state, autonomic dysfunction, and an elevated CK level. Particularly in a patient with a history of NMS, a thorough medication history is essential to aid diagnosis and avoid confusion with presenting symptoms and medical history.


    Institute of Scientific and Technical Information of China (English)

    WANG Hong-xing; LV Qiu-lin; WANG Zu-cheng; HUANG Ji-zhong; XU He-ding; WEN Hui; CHEN Jun; QIAO Song; NI Xiao-dong


    Objective To report one case of neuroleptic malignant syndrome (NMS) with raising blood sugar. Methods The patient was studied clinically with biochemistry, white blood cells, psychiatric symptoms,and creatine phosphokinase (CPK) observations. Results The male patient with a history of taking antipsychotics more 30 years and his age of onset was about 20 years. He had severe muscular rigidity, altered consciousness and autonomic disturbance associated with elevation of serum CPK levels ( max 3755 U/L) and leucocytosis ( max 13.3 × 109/L ) , especially granular leukocytosis( max 90% ) and lymphocytopenia (min 8% ). In addition, high blood sugar emerged along with the variation of white blood cells ( max 9. 0 mmol/L). Conclusion The manifestations of the patient was in conformity with those of the NMS. The patient had catatonic signs such as mutism,drinking difficulty, etc. and excess of saliva. Developmental observation with CPK and white blood cells is able to reveal the severity level of NMS. Raising blood sugar should be also monitoring item.

  7. Prevalence of goitre among school going children in urban area of Dehradun

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    Utkarsh Sharma


    Full Text Available Background: Iodine is an important micro-nutrient required for human nutrition. Iodine Deficiency Disorders (IDDs are one of the major world-wide public health problems of today which causes wide spectrum of disabilities. It includes impairment of reproductive functions, lowering of IQ levels in school age children, goiter, deaf mutism, mental defects, weakness and paralysis of muscles as well as lesser degree of physical dysfunction. Methods: Selection of population: The school children in age group of 6-18 years from both the sexes were screened from SGRR Schools of different locations at Dehradun, after taking approval from principal and the parents. Results: The prevalence of goitre among school going children was 5%. Prevalence of goitre among female was 6.4% compare to male were 4.1%. There was significant association found between prevalence of goitre and vegetarian diet. In pre pubertal age (11-14 years maximum (7.6% cases of goitre were seen. A significant association of goitre with pallor was also observed Conclusion: The sustained efforts in implementing the guidelines of National Iodine Deficiency Disorders Control Programme (NIDDCP have been able to reduce the prevalence of goitre in Uttrakhand state. In spite of reduction in prevalence over years, goitre continues to be a major public health problem in the state. [Int J Res Med Sci 2015; 3(1.000: 198-200


    Directory of Open Access Journals (Sweden)

    Akbari V J


    Full Text Available The fourth ventricle enlarges in various conditions like hydrocephalus, dementias, mutism etc. So, it is necessary to know the normal range of dimensions of forth ventricle. Present study was conducted at anatomy Dept. P.D.U. medical college, Rajkot, Gujarat during October 2009 to October 2010. The casts of ventricular system of 20 formalinized cadaveric human brains were prepared after injecting epoxyresin+hardener (BOND TITE mixture. From the cast, measurements of height (from upper end of impression of superior medullary valum to obex and breadth (at the level of lateral recesses of 4th ventricle were taken by vernier caliper. The data was analyzed statistically and was compared with the data obtained from radiological methods. In present study, the mean height of fourth ventricle 2.29 cm (range 1.9-2.7cm, S.D. 0.30 and mean breadth 2.38 cm (range 1.75-3 cm, S.D. 0.44. Present data differs significantly from data of radiological studies, which could be due to different methodologies, whereas the radiological methods are indirect and two dimensional; the present study with cast gives direct and three dimensional results without distorting the dimensions of fourth ventricle. [National J of Med Res 2011; 1(2.000: 48-50

  9. Antiretroviral treatment induced catatonia in 16-year-old boy

    Directory of Open Access Journals (Sweden)

    Anand Lingeswaran


    Full Text Available We present a 16-year-old boy, who had presented to us with catatonic features of mutism, withdrawal, passive negativism, grimacing, gesturing, echopraxia, and excitement of 5 days duration while taking antiretroviral therapy (ART for a period of 2 years. He had history of birth asphyxia and acquired HIV infection from his father when the same syringe and needle was used on both of them in a medical setting where the father and son had consulted for treatment of pyrexia of unknown origin. He was the eldest of a three children family in which the biologic father had acquired HIV through extramarital sexual contact with HIV-infected sex workers but was unaware of his HIV positive status till our patient, the 16-year-old was admitted and treated for pulmonary tuberculosis at 14 years of age. The boy′s mother had only acquired HIV after having three children with the HIV-positive husband, thus leaving the other two children HIV negative. The catatonia completely resolved within 2 days after the ART was withheld, and risperidone 1 mg twice a day was prescribed. This case highlights the risks of ART and breach of universal precautions.

  10. Wax on, wax off: a rare case of catatonia. (United States)

    Greenberg, Karen; D'Ambrosio, Michael; Liebman, Kenneth M; Veznedaroglu, Erol


    Catatonia was first described by a German psychiatrist, Karl Kahlbaum, in 1874. It is a behavioral syndrome marked by an inability to move normally, which can occur in the context of many underlying general medical and psychiatric disorders. A wide variety of neurologic, metabolic, drug-induced, and psychiatric causes of catatonia have been reported. We present a unique case of late onset catatonia in a 56-year-old man with no prior medical or psychiatric history initially presenting with stroke-like symptoms. The patient was awake and alert, with spontaneous eye opening, but completely nonverbal and not following any commands. Specifically, the patient demonstrated stupor, catalepsy, mutism, and negativism. After extensive emergency department testing, including negative computed tomography head, negative magnetic resonance imaging brain, negative electroencephalogram, and normal laboratory results, the patient was diagnosed with new-onset bipolar disorder with depressive features presenting as catatonia. Recognizing catatonia is important because it may be caused or exacerbated by treatment of the underlying disorder. Failure to institute treatment early in the course of catatonia is associated with a poor prognosis.

  11. Catatonia in patients with autism: prevalence and management. (United States)

    Mazzone, Luigi; Postorino, Valentina; Valeri, Giovanni; Vicari, Stefano


    Although recent studies have shown that catatonia can occur in patients with autism spectrum disorders (ASDs), the overlap of the behavioral features between these disorders raises many diagnostic challenges. In fact, in clinical practice it is common to misinterpret catatonic symptoms, including mutism, stereotypic speech, repetitive behaviors, echolalia, posturing, mannerisms, purposeless agitation and rigidity, as features of ASDs. The current medical treatment algorithm for catatonia in ASDs recommends the use of benzodiazepines. Electroconvulsive therapy (ECT) is indicated when patients are unresponsive, or insufficiently responsive, to benzodiazepines. Other pharmacological options are also described for the treatment of catatonic patients resistant to benzodiazepines and ECT, and there is evidence for the effectiveness of a psychological treatment, co-occurring with medical treatments, in order to support the management of these patients. In this article we provide a summary of studies exploring catatonia in ASDs and our clinical experience in the management and treatment of this syndrome through the presentation of three brief case studies. Moreover, we review the mechanisms underlying symptoms of catatonia in ASDs, as well as the diagnostic challenges, providing an outline for the management and treatment of this syndrome in this clinical population.

  12. Catatonia: Our current understanding of its diagnosis, treatment and pathophysiology (United States)

    Rasmussen, Sean A; Mazurek, Michael F; Rosebush, Patricia I


    Catatonia is a psychomotor syndrome that has been reported to occur in more than 10% of patients with acute psychiatric illnesses. Two subtypes of the syndrome have been identified. Catatonia of the retarded type is characterized by immobility, mutism, staring, rigidity, and a host of other clinical signs. Excited catatonia is a less common presentation in which patients develop prolonged periods of psychomotor agitation. Once thought to be a subtype of schizophrenia, catatonia is now recognized to occur with a broad spectrum of medical and psychiatric illnesses, particularly affective disorders. In many cases, the catatonia must be treated before any underlying conditions can be accurately diagnosed. Most patients with the syndrome respond rapidly to low-dose benzodiazepines, but electroconvulsive therapy is occasionally required. Patients with longstanding catatonia or a diagnosis of schizophrenia may be less likely to respond. The pathobiology of catatonia is poorly understood, although abnormalities in gamma-aminobutyric acid and glutamate signaling have been suggested as causative factors. Because catatonia is common, highly treatable, and associated with significant morbidity and mortality if left untreated, physicians should maintain a high level of suspicion for this complex clinical syndrome. Since 1989, we have systematically assessed patients presenting to our psychiatry service with signs of retarded catatonia. In this paper, we present a review of the current literature on catatonia along with findings from the 220 cases we have assessed and treated. PMID:28078203

  13. The Wandering Woman: A Case Study of Catatonia vs Factitious Disorder (United States)

    Wong, Jennifer WH; Williams, Steven R


    A 61-year-old woman with an unknown psychiatric history presented with mutism, stupor, negativism, and withdrawn behavior. She was admitted to the psychiatric unit for what appeared to be catatonia. Medical records were not readily available. A comprehensive evaluation did not uncover any medical etiology. Lorazepam was ineffective at consistently reversing her catatonic symptoms. During week three of hospitalization, she was given olanzapine with subsequent improvement in her negativism. Several physicians believed her catatonic symptoms were feigned given multiple episodes of spontaneous purposeful movement when she was not under the direct supervision of staff. There is minimal literature on distinguishing catatonia and factitious disorder. This distinction is crucial because these diagnoses require very different treatments, and the iatrogenic complications related to the treatment of catatonia with high-dose benzodiazepines and electroconvulsive therapy are significant. Rapid access to electronic health records can facilitate treatment for patients who cannot provide a medical history, especially when factitious disorder is included in the differential diagnosis. PMID:28352494

  14. Special medical conditions associated with catatonia in the internal medicine setting: hyponatremia-inducing psychosis and subsequent catatonia. (United States)

    Novac, Andrei A; Bota, Daniela; Witkowski, Joanne; Lipiz, Jorge; Bota, Robert G


    Diagnosis and treatment of catatonia in the psychiatry consultation service is not infrequent. Usually, the patient either presents to the Emergency Department or develops catatonia on the medical floor. This condition manifests with significant behavioral changes (from mildly decreased speech output to complete mutism) that interfere with the ability to communicate. After structural brain disorders are excluded, one of the diagnoses that always should be considered is catatonia. However, the causes of catatonia are numerous, ranging from psychiatric causes to a plethora of medical illnesses. Therefore, it is not surprising that there are many proposed underlying mechanisms of catatonia and that controversy persists about the etiology of specific cases.There are only 6 reports of hyponatremia-induced catatonia and psychosis in the literature. Here, we present the case of a 30-year-old woman with catatonia and psychosis induced by hyponatremia, and we use this report to exemplify the multitude of biologic causes of catatonia and to propose a new way to look at the neuroanatomical basis of processing, particularly the vertical processing systems we believe are involved in catatonia.

  15. Catatonia: Our current understanding of its diagnosis, treatment and pathophysiology. (United States)

    Rasmussen, Sean A; Mazurek, Michael F; Rosebush, Patricia I


    Catatonia is a psychomotor syndrome that has been reported to occur in more than 10% of patients with acute psychiatric illnesses. Two subtypes of the syndrome have been identified. Catatonia of the retarded type is characterized by immobility, mutism, staring, rigidity, and a host of other clinical signs. Excited catatonia is a less common presentation in which patients develop prolonged periods of psychomotor agitation. Once thought to be a subtype of schizophrenia, catatonia is now recognized to occur with a broad spectrum of medical and psychiatric illnesses, particularly affective disorders. In many cases, the catatonia must be treated before any underlying conditions can be accurately diagnosed. Most patients with the syndrome respond rapidly to low-dose benzodiazepines, but electroconvulsive therapy is occasionally required. Patients with longstanding catatonia or a diagnosis of schizophrenia may be less likely to respond. The pathobiology of catatonia is poorly understood, although abnormalities in gamma-aminobutyric acid and glutamate signaling have been suggested as causative factors. Because catatonia is common, highly treatable, and associated with significant morbidity and mortality if left untreated, physicians should maintain a high level of suspicion for this complex clinical syndrome. Since 1989, we have systematically assessed patients presenting to our psychiatry service with signs of retarded catatonia. In this paper, we present a review of the current literature on catatonia along with findings from the 220 cases we have assessed and treated.

  16. ECT in the treatment of a patient with catatonia: consent and complications. (United States)

    Zisselman, Marc H; Jaffe, Richard L


    Acute catatonia in an adolescent or young adult can present complex clinical challenges. Prominent issues include those involving diagnosis, timely and effective treatment, and diminished capacity to provide consent. The authors describe a 19-year-old woman presenting initially with manic excitement followed by a lengthy period of mutism, immobility, and food and fluid refusal. Elevated temperature, an elevated creatine phosphokinase level, and autonomic dysfunction led to consideration of a malignant catatonic syndrome. The patient manifested rigidity accompanied by posturing and waxy flexibility. Neurologic, medical, and laboratory evaluations failed to identify an organic cause for the likely catatonia. Treatment with amantadine, bromocriptine, and lorazepam was unsuccessful. ECT was deemed appropriate but required emergency guardianship because of the patient's inability to provide consent. At the initial ECT session, the elicited seizure was followed by an episode of torsade de pointes requiring immediate cardioversion. In reviewing the ECT complication, it appeared that muscle damage due to catatonic immobility led to acute hyperkalemia with the administration of succinylcholine. Discussions were held with the patient's guardian outlining the clinical issues and the risks of additional ECT. The patient responded to eight subsequent ECT sessions administered with rocuronium, a nondepolarizing muscle relaxant. The authors provide a brief review of the diagnosis and treatment of catatonia and address issues surrounding ECT, cardiac effects, use of muscle relaxants, and the consent process.

  17. [Recovery of consciousness: process-oriented approach]. (United States)

    Gusarova, S B


    Traditionally psychological neurorehabilitation of neurosurgical patients is provided subject to availability of clear consciousness and minimal potential to communicate verbally. Cognitive and emotional disorders, problems in social adaptation, neurotic syndromes are normally targets in such cases. We work with patients having survived severe brain damage being in different states of consciousness: vegetative state, minimal state of consciousness, mutism, confusion, posttraumatic Korsaroff syndrom. Psychologist considers recovery of consciousness as the target besides traditional tasks. Construction of communication with patient is central part of such job, where the patient remains unable to contact verbally, yet it is impossible to consider potential aphasia. This is a non-verbal "dialogue" with patient created by psychologist with gradual development and involving other people and objects of environment. Inline with modern neuroscientific achievements demonstrating ability to recognize by patients with severe brain injury (A. Owen, S. Laureys, M. Monti, M. Coleman, A. Soddu, M. Boly and others) we base upon psychological science, on psychotherapeutic approaches containing instruments inevitable to work with patients in altered states of consciousness and creation of non-verbal communication with patient (Jung, Reich, Alexander, Lowen, Keleman, Arnold and Amy Mindell, S. Tomandl, D. Boadella, A. Längle, P. Levin etc). This article will include 15 years of experience to apply Process-oriented approach by A. Mindell to recovery of consciousness of neurosurgical patients based on work with "minimal signals" (micro moves, breath, mimic reactions etc.), principle of feedback, psychosomatic resonance, empathy.

  18. Global aphasia as a predictor of mortality in the acute phase of a first stroke

    Directory of Open Access Journals (Sweden)

    F F Oliveira


    Full Text Available OBJECTIVE: To establish whether vascular aphasic syndromes can predict stroke outcomes. METHOD: Thirty-seven adults were evaluated for speech and language within 72 hours after a single first-ever ischemic brain lesion, in blind association to CT and/or MR. RESULTS: Speech or language disabilities were found in seven (87.5% of the eight deceased patients and twenty-six (89.7% of the twenty-nine survivors. Global aphasia was identified in eleven patients, all with left hemisphere lesions (nine mute; five deceased, consisting on a risk factor for death in the acute stroke phase (ρ=0.022. Age (z=1.65; ρ>0.09, thrombolysis (ρ=0.591, infarct size (ρ=0.076 and side (ρ=0.649 did not significantly influence survival. Absence of aphasia did not predict a better evolution, regardless of the affected hemisphere. Prevalence of cardiovascular risk factors was similar for all patient groups. CONCLUSION: Global aphasia in acute stroke can adversely affect prognosis, translated into impairment of dominant perisylvian vascular territories, with mutism as an important semiological element.

  19. [A psycholinguistic study in a patient with echolalia]. (United States)

    Diesfeldt, H F


    Language is often impaired in patients with a primary degenerative dementia syndrome. Severe dementia may even lead to mutism, a condition in which the person does not produce any spontaneous speech, despite preservation of consciousness. The question is if there are any language abilities left in patients who do not speak. In this single case-study the echolalic behaviour of a patient with severe dementia and mixed transcortical aphasia (or isolation of the speech area) was analysed by means of special linguistic tasks. In repeating sentences violating number agreement, the patient spontaneously corrected the pronoun, the noun or the verb. Furthermore, her verbal output during repetition tasks was highly constrained to lexically acceptable Dutch nouns. Despite extremely poor comprehension, this patient did not repeat verbal utterances in a parrot-like fashion. There are not many reports in the literature of patients who develop a mixed transcortical aphasia as a consequence of primary degenerative dementia. A comparable case was described by H. Whitaker. Single case-studies, such as these, are essential to our insight into the course of language breakdown in senile dementia. The preserved repetition ability of this patient demonstrates the robustness of the articulatory loop system, even if there is a severe comprehension deficit.

  20. Management of developmental speech and language disorders. Part 2: acquired conditions. (United States)

    O'Hare, Anne


    Many children who present with these acquired impairments of communication have a clear preceding event such as an acquired brain injury from a road traffic accident. Children often respond differently in this situation to adult presentations. They may have a period of mutism when the prognosis might look poor and yet they subsequently make rapid progress and recover speech. They have greater potential for neural plasticity and language recovery, although they often have persisting difficulties in oral and written language. Alternatively, there may be a presentation with a paroxysmal event such as a seizure or a period of depressed consciousness, and the unusual behaviour that may accompany dysphasia and dysarthria may be misinterpreted in the child, whereas for the adult with the more common 'stroke-like' presentation, it would be immediately considered. Rarely the aphasia/dysphasia may itself be the paroxysmal event where actually recognising that the child's disrupted communication is the basis of any observed behaviours can be the greater challenge.

  1. Afebrile Neuroleptic Malignant Syndrome associated with Fluphenazine decanoate: A case report

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    Marzieh Assareh


    Full Text Available "nNeuroleptic Malignant Syndrome (NMS is unusual but could be a lethal reaction associated with neuroleptic drugs. It occurs in almost 0.07-2.2% of patients under treatment with neuroleptics. There are some medical treatments that may also be helpful for its treatment, including dopamine agonists, muscle relaxants, and electroconvulsive therapy (ECT. We present this case to alert the clinicians to the potential for inducing afebrile NMS. Our case is a 41-year-old man with a history of schizophrenia showing signs and symptoms in accordance with NMS, 2 weeks after receiving one dose of 12.5 mg fluphenazine decanoate, abruptly following the 3rdsession of ECT. The patient presented with decreased level of consciousness, muscular rigidity, waxy flexibility, mutism ,generalized tremor, sever diaphoresis and tachycardia which progressed during the previous 24 h. Laboratory data indicated primarily leukocytosis, an increasing level of creatinine phosphokinase and hypokalemia during the next 72h. In patients receiving antipsychotics, any feature of NMS should carefully be evaluated whether it is usual or unusual particularly in patients receiving long acting neuroleptics.

  2. Dysgraphia in patients with primary lateral sclerosis: a speech-based rehearsal deficit? (United States)

    Zago, S; Poletti, B; Corbo, M; Adobbati, L; Silani, V


    The present study aims to demonstrate that errors when writing are more common than expected in patients affected by primary lateral sclerosis (PLS) with severe dysarthria or complete mutism, independent of spasticity. Sixteen patients meeting Pringle's et al. [34] criteria for PLS underwent standard neuropsychological tasks and evaluation of writing. We assessed writing abilities in spelling through dictation in which a set of words, non-words and short phrases were presented orally and by composing words using a set of preformed letters. Finally, a written copying task was performed with the same words. Relative to controls, PLS patients made a greater number of spelling errors in all writing conditions, but not in copy task. The error types included: omissions, transpositions, insertions and letter substitutions. These were equally distributed on the writing task and the composition of words with a set of preformed letters. This pattern of performance is consistent with a spelling impairment. The results are consistent with the concept that written production is critically dependent on the subvocal articulatory mechanism of rehearsal, perhaps at the level of retaining the sequence of graphemes in a graphemic buffer. In PLS patients a disturbance in rehearsal opportunity may affect the correct sequencing/assembly of an orthographic representation in the written process.

  3. Neuronal Control of Mammalian Vocalization, with Special Reference to the Squirrel Monkey (United States)

    Jürgens, Uwe

    Squirrel monkey vocalization can be considered as a suitable model for the study in humans of the neurobiological basis of nonverbal emotional vocal utterances, such as laughing, crying, and groaning. Evaluation of electrical and chemical brain stimulation data, lesioning studies, single-neurone recordings, and neuroanatomical tracing work leads to the following conclusions: The periaqueductal gray and laterally bordering tegmentum of the midbrain represent a crucial area for the production of vocalization. This area collects the various vocalization-triggering stimuli, such as auditory, visual, and somatosensory input from diverse sensory-processing structures, motivation-controlling input from some limbic structures, and volitional impulses from the anterior cingulate cortex. Destruction of this area causes mutism. It is still under dispute whether the periaqueductal region harbors the vocal pattern generator or merely couples vocalization-triggering information to motor-coordinating structures further downward in the brainstem. The periaqueductal region is connected with the phonatory motoneuron pools indirectly via one or several interneurons. The nucleus retroambiguus represents a crucial relay station for the laryngeal and expiratory component of vocalization. The articulatory component reaches the orofacial motoneuron pools via the parvocellular reticular formation. Essential proprioceptive feedback from the larynx and lungs enter the vocal-controlling network via the solitary tract nucleus.

  4. Anxiety disorders in children%儿童的焦虑障碍

    Institute of Scientific and Technical Information of China (English)



    儿童的焦虑障碍是常见的精神障碍,常与躯体症状或躯体疾病相伴随,总体的终生患病率较高.儿童焦虑障碍主要包括分离性焦虑障碍、恐惧性焦虑障碍、社交性焦虑障碍、广泛性焦虑障碍、惊恐障碍和选择性缄默.现主要介绍上述5种焦虑障碍的流行病学、病因与病理机制、常见临床表现、评估、治疗和预后.%Child anxiety disorders are among the common mental health disorders,often with physical symptoms or physical disorders.The life time prevalence is relative high.Child anxiety disorders mainly include in subtype as separation anxiety,specific phobia,social anxiety disorder,panic disorder and selective mutism.In this paper,the prevalence rate of the above 5 subtypes,etiology,clinic manifestation,treatment and outcome were introduced.

  5. Pick and Alzheimer diseases: a rare comorbidity presenting as corticobasal syndrome. (United States)

    Rusina, Robert; Pazdera, Ladislav; Kulišťák, Petr; Vyšata, Oldřich; Matěj, Radoslav


    We describe a patient with corticobasal syndrome in whom neuropathological examination on autopsy revealed Pick and Alzheimer diseases in comorbidity. Corticobasal degeneration is a tauopathy usually associated with asymmetric parkinsonism, parietal lobe involvement, and cognitive impairment. Corticobasal syndrome is the clinical presentation of corticobasal degeneration without neuropathological confirmation. A 66-year-old right-handed man slowly developed speech difficulties, right-hand clumsiness, and forgetfulness. His speech apraxia progressed to mutism with preserved comprehension, and his clumsiness progressed to severe apraxia involving both hands. He developed behavioral changes and severe amnesia. All of these features were consistent with corticobasal syndrome. His loss of episodic, verbal, and visuospatial memory suggested Alzheimer disease; however, beyond his frontotemporal neuropsychological profile, he had few symptoms characteristic of frontal lobe dementia. Magnetic resonance imaging scans showed worsening temporal, frontal, and parietal atrophy, predominant in the left hemisphere. Neuropathological examination at autopsy revealed abundant neuritic plaques and neurofibrillary tangles consistent with fully developed Alzheimer disease, as well as numerous intraneuronal Pick bodies in the frontotemporal lobes. Our findings confirm the importance of clinical and neuropathological correlations in patients with atypical neurodegenerative dementias.

  6. Role of early audiological screening and intervention

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    Isha Preet Tuli


    Full Text Available Objectives: The aim of our study is to find out whether early identification and intervention prevents severe linguistic and communication delays, minimizing the need for more elaborate rehabilitation during the school years. Design and Study Sample: A Cross-sectional study was carried out on one hundred and eleven children with documented deaf-mutism presenting at the Department of Oto-Rhino-Laryngology in two different geographical regions of India. Detailed history, clinical and audiological assessment along with assessment of the language and communication skills according to American Speech Language Hearing Association (ASHA guidelines was done. Results: The children were predominantly male, of low socio-economic background, presented for rehabilitation, mostly after the age of six years. They commonly had more than 70dBHL (decibel hearing level hearing threshold. The study found that the children who presented early for diagnosis and hearing aid usage had better response to rehabilitation. Conclusion: It is important that hearing loss be detected at an early age and amplification should be introduced as early as possible once there has been a diagnosis of a hearing loss. So that early intervention can have an impact on speech and language development. Without concurrent follow-up program, identification of hearing loss early in children is meaningless.

  7. Catatonia in disulfiram intoxication - a case report and a brief overview of the literature. (United States)

    Takacs, Rozalia; Milan, Flora; Ungvari, Gabor S; Faludi, Gabor; Gazdag, Gabor


    Catatonic syndromes could accompany a variety of psychiatric and medical conditions. The most common conditions underlying catatonia are affective disorders followed by schizophrenia, but several medical conditions including intoxications affecting the central nervous system can also present with catatonic signs and symptoms. Therapeutic doses of disulfiram could induce catatonia with or without accompanying psychosis or mood disorder. A case of disulfiram intoxication manifesting with catatonia is reported here together with a brief overview of the literature. A patient was admitted to the toxicology ward after a suicide attempt with approximately 20 g of disulfiram. On transfer to the psychiatric ward, she was sitting still, in a semi-stuporous state and displayed motiveless resistance to instructions or attempts to move (active negativism). She was unresponsive to most of the questions (mutism), occasionally verbigerated 1-2 words and stared for more than 20 seconds between shifting attention. After developing a comatosus state her treatment continued at the toxicology ward, where a contrast-enhanced computer tomography scan revealed bilateral emollition of 1.5 cm diameter in both nucleus lentiformis at the level of the third ventricle. Following treatment her condition improved and she benefited of rehabilitation facility and a second psychiatric treatment. She was discharged free of neurological and psychiatric symptoms. In conclusion, we underscore the importance of accurate diagnosis of the underlying psychiatric or medical condition when encountering a fast emerging catatonic syndrome and focus first on treating the causative condition while simultaneously attempting symptomatic treatment of catatonia.

  8. Complete clinical recovery of a central pontine and extrapontine myelinolysis delayed onset in a child with acute myeloblastic leukemia. (United States)

    Yilmaz, D; Karapinar, B; Balkan, C; Ay, Y; Kavakli, K


    Central pontine myelinolysis (CPM) is a demyelinating disease of the pons often associated with the demyelination of extrapontine areas of the central nervous system. It typically occurs 0.5-7 days after a rapid increment in serum Na level in hyponatremic patients and may lead to death. A 2.5-year-old child with a diagnosis of acute myeloblastic leukemia developed febril neutropenia, diarrhea, gastrointestinal hemorrhage followed by pulmonary aspergillosis. He could not tolerate enteral nutrition. He was given broad spectrum antibiotics and antifungal treatment. Laboratory tests showed electrolyte abnormalities including hyponatremia, hypokalemia and hypophosphatemia in a chronic course. Twenty three days after a rapid correction of hyponatremia (16 mEq/L/24 h) he revealed flask quadriparesis, disphagia, mutism, irregular respiratory pattern and loss of cough and gag reflex. Cranial magnetic resonance showed central pontine and extrapontine myelinolysis. He required mechanical ventilation and then he regained his neurologic functions. He completed chemotherapy protocol and underwent hematopoietic stem cell transplantation. After 2.5 years of the occurrence of CPM he is in completely normal physical and neurological status. CPM is a very severe but rare disorder in children with underlying disease. In the presence of multiple etiologic factors it may reveal a delayed onset and optimum outcome can be seen even in the severe clinical presentation with adequate intensive support.

  9. Differences in striatal dopamine transporter density between tremor dominant and non-tremor Parkinson's disease

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    Kaasinen, Valtteri; Kinos, Maija; Joutsa, Juho [University of Turku and Turku University Hospital, Division of Clinical Neurosciences, Turku (Finland); University of Turku and Turku University Hospital, Turku PET Centre, Turku (Finland); Seppaenen, Marko [University of Turku and Turku University Hospital, Turku PET Centre, Turku (Finland); University of Turku and Turku University Hospital, Department of Clinical Physiology and Nuclear Medicine, Turku (Finland); Noponen, Tommi [University of Turku and Turku University Hospital, Department of Clinical Physiology and Nuclear Medicine, Turku (Finland)


    Parkinson's disease (PD) can manifest with a tremor-dominant or a non-tremor (akinetic-rigid) phenotype. Although the tremor-dominant subtype may show a better prognosis, there is limited information on the phenotypic differences regarding the level of striatal dopamine transmission. The present study investigated striatal dopamine transporter (DAT) binding characteristics in a large sample of patients with and without tremor. [{sup 123}I]FP-CIT SPECT scans of 231 patients with a clinical diagnosis of PD and abnormal FP-CIT binding (157 with tremor, 74 without tremor) and 230 control patients with normal FP-CIT binding (148 with tremor, 82 without tremor) were analysed using an automated region-of-interest analysis of the scans (BRASS). Specific striatal binding ratios were compared between phenotypes and groups using age, sex, and symptom duration, predominant side of symptoms, dopaminergic medications and scanner as covariates. Patients with PD had 28.1 - 65.0 % lower binding in all striatal regions compared to controls (p < 0.001). The mean FP-CIT caudate nucleus uptake and the left caudate nucleus uptake were higher in PD patients with tremor than in PD patients without tremor (mean 9.0 % higher, left 10.5 % higher; p < 0.05), whereas there were no differences between tremor and non-tremor control patients. No significant effects of tremor on DAT binding were observed in the anterior or posterior putamen. The motor phenotype is associated with the extent of caudate dopamine terminal loss in PD, as dopamine function is relatively more preserved in tremor patients. Symptom type is related to caudate dopamine function only in association with Parkinsonian dopaminergic degeneration, not in intact dopamine systems in patients with non-PD tremor. (orig.)

  10. A Comparison between Quantitative Gated Myocardial Perfusion Scintigraphy and Strain Echocardiography as Indicators of Ventricular Functions in Patients with Anterior Myocardial Infarction (United States)

    Karacavus, Seyhan; Celik, Ahmet; Tutus, Ahmet; Kula, Mustafa; Oguzhan, Abdurrahman; Ozdogru, Ibrahim; Kalay, Nihat


    The purpose of this study is to compare the strain echocardiographic and scintigraphic parameters for evaluating of the left ventricular (LV) functions in patients with anterior myocardial infarction (MI). Fifty-four patients (male/female: 36/18; mean age 62 ± 13 years) with anterior MI were prospectively enrolled. All patients were performed gated myocardial perfusion scintigraphy gated single-photon emission computed tomography (GSPECT) and echocardiography (EC). GSPECT data were processed and analyzed using 4D-MSPECT (4DM, Invia Medical Imaging Solutions, Ann Arbor, MI, USA). The echocardiographic strain (S) and strain rate (SR) values were calculated. The results obtained by these techniques were compared each other. A total of 918 segments of LV wall were evaluated. In all patients, 385 segments were automatically scored as normokinetic, 206 as hypokinetic, 122 as akinetic, 205 as dyskinetic and 300 as normal thickening, 348 as decrease thickening and 270 as no thickening. The means of S and SR values in thickening and motion score groups according to GSPECT were statistically different from each other (P < 0.001). There was a negative significant correlation between LV wall thickening sum score and S and SR and between LV wall motion sum score and S and SR (P < 0.001). There was a good correlation between GSPECT and echocardiographic LV-ejection fraction (r = 0.7, P < 0.001). GSPECT and strain EC are similar in quantitative grading of the severity of regional and global myocardial dysfunction in patients with anterior MI and these techniques provide valuable diagnostic information. PMID:25538490

  11. Iodine 123-labeled meta-iodobenzylguanidine myocardial scintigraphy in the cases of idiopathic Parkinson`s disease, multiple system atrophy, and progressive supranuclear palsy

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    Yoshita, Mitsuhiro; Hayashi, Michiyuki; Hirai, Shunsaku [Tokyo Metropolitan Neurological Hospital (Japan)


    To investigate cardiac sympathetic function in Parkinson`s disease (PD), multiple system atrophy (MSA), and progressive supranuclear palsy (PSP), {sup 123}I-MIBG myocardial scintigraphy was performed in 25 patients with PD, 25 patients with MSA, 14 patients with PSP, and 20 control subjects. In planar imaging studies, the heart-to-mediastinum average count ratio (H/M) was calculated for both early and delayed images. The mean value of H/M in patients with PD was significantly lower than in those with MSA, PSP, or no disease. Regardless of disease severity or intensity of anti-parkinsonian pharmacotherapy, mean values for H/M were always low in patients with PD. The mean values of H/M in patients with MSA and PSP were significantly lower than in controls. There was no significant difference between the mean value of H/M in MSA with orthostatic hypotension (OH) and that in MSA without OH, and also there was no significant difference between the mean value of H/M in MSA with striatonigral degeneration and that in MSA with olivopontocerebellar atrophy. Although the mean value of H/M in PSP with amitriptyline treatment was significantly lower than that in PSP patients without amitriptyline treatment, there was no significant difference between the mean value of H/M in PSP patients without amitriptyline treatment and that in controls. There was no correlation between H/M and disease duration in those three akinetic-rigid disorders that we have studied here. Thus, PD may have an abnormality of cardiac sympathetic function which has not been detected by previous cardiovascular autonomic studies. Particularly in early stages, {sup 123}I-MIBG myocardial scintigraphy may help to differentiate PD from MSA and PSP. (K.H.)

  12. The Effect of Sleep and Wakefulness Disorders on Cognitive Function in Parkinson's Disease

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    Rustambek Matmurodov


    Full Text Available Objective: To clarify clinical features of sleep disorders in patients with Parkinson’s disease (PD and their effect on cognitive function and emotional-personal sphere. Material and methods: The study involved 62 patients with PD (33 men and 29 women mean age 47.8+7.1 years. The control group consisted of 20 patients without Parkinsonism matching by age and sex. The diagnosis of PD was established by criteria A.Hughes. Patients underwent an extended neuropsychological study with qualitative and quantitative analysis. To determine cognitive disorders, we used neuropsychological tests. Results: 49 patients (79,3% had sleep disorders. In the structure of sleep disorders: 29 (59.2% patients had insomnia and 11 (22.4% patients – parasomnia and 9 (18.4% - hypersomnia. In a control group, numbers were 4.5 times less. The analysis showed that the structure of sleep disorders correlates with the form of PD. So, insomnia is more rapidly met in akinetic-rigid form (55.5%, whereas hypersomnia is presented in mixed form. Patients with insomnia present 35% of predement cognitive impairment and 34% of dementia (66.7% of mild degree dementia and 33.3% moderate dementia severity. Whereas, in patients with hypersomnia 35% of predement cognitive impairment, 60% of dementia (54.6% and 45.4% were observed. Among patients with permanent drowsiness 32.4% of predement cognitive impairment and 58.4% of dementia (44.8% and 55.2% were diagnosed. Dementia (65.4% was observed more in patients with sudden sleep (42,8% and 57.2%. Conclusion: sleep and wakefulness disorders depend not only on the severity of motor symptoms, but also depend on the emotional and cognitive state.

  13. Identification by [{sup 99m}Tc]ECD SPECT of anterior cingulate hypoperfusion in progressive supranuclear palsy, in comparison with Parkinson's disease

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    Varrone, Andrea [University Federico II, Biostructure and Bioimaging Institute, National Research Council/Department of Biomorphological and Functional Sciences, Napoli (Italy); Karolinska Hospital, Department of Clinical Neuroscience, Psychiatry Section, Stockholm (Sweden); Pagani, Marco; Salmaso, Dario [National Research Council, Institute of Cognitive Sciences and Technologies, Rome and Padua (Italy); Salvatore, Elena; Amboni, Marianna; De Michele, Giuseppe; Filla, Alessandro; Barone, Paolo [University Federico II, Department of Neurological Sciences, Napoli (Italy); Sansone, Valeria; Pappata, Sabina; Salvatore, Marco [University Federico II, Biostructure and Bioimaging Institute, National Research Council/Department of Biomorphological and Functional Sciences, Napoli (Italy); Nobili, Flavio [University of Genoa, Clinical Neurophysiology, Department of Endocrinological and Metabolic Sciences, Genoa (Italy)


    Progressive supranuclear palsy (PSP) is an akinetic-rigid syndrome that can be difficult to differentiate from Parkinson's disease (PD), particularly at an early stage. [{sup 99m}Tc]ethyl cysteinate dimer (ECD) SPECT could represent a widely available tool to assist in the differential diagnosis. In this study we used voxel-based analysis and Computerised Brain Atlas (CBA)-based principal component analysis (PCA) of [{sup 99m}Tc]ECD SPECT data to test whether: (1) specific patterns of rCBF abnormalities can differentiate PSP from controls and PD; (2) networks of dysfunctional brain regions can be found in PSP vs controls and PD. Nine PD patients, 16 PSP patients and ten controls were studied with [{sup 99m}Tc]ECD SPECT using a brain-dedicated device (Ceraspect). Voxel-based analysis was performed with statistical parametric mapping. PCA was applied to volume of interest data after spatial normalisation to CBA. The voxel-based analysis showed hypoperfusion of the anterior cingulate and medial frontal cortex in PSP compared with controls and PD. In PSP patients the rCBF impairment extended to the pre-supplementary motor area and prefrontal cortex, areas involved in executive function and motor networks. Compared with PSP patients, PD patients showed a mild rCBF decrease in associative visual areas which could be related to the known impairment of visuospatial function. The PCA identified three principal components differentiating PSP patients from controls and/or PD patients that included groups of cortical and subcortical brain regions with relatively decreased (cingulate cortex, prefrontal cortex and caudate) or increased (parietal cortex) rCBF, representing distinct functional networks in PSP. Anterior cingulate hypoperfusion seems to be an early, distinct brain abnormality in PSP as compared with PD. (orig.)

  14. Morphology and elemental composition of recent and fossil cyanobacteria (United States)

    St. Amand, Ann; Hoover, Richard B.; Jerman, Gregory A.; Coston, James; Rozanov, Alexei Y.


    Cyanobacteria (cyanophyta, cyanoprokaryota, and blue-green algae) are an ancient, diverse and abundant group of photosynthetic oxygenic microorganisms. Together with other bacteria and archaea, the cyanobacteria have been the dominant life forms on Earth for over 3.5 billion years. Cyanobacteria occur in some of our planets most extreme environments - hot springs and geysers, hypersaline and alkaline lakes, hot and cold deserts, and the polar ice caps. They occur in a wide variety of morphologies. Unlike archaea and other bacteria, which are typically classified in pure culture by their physiological, biochemical and phylogenetic properties, the cyanobacteria have historically been classified based upon their size and morphological characteristics. These include the presence or absence of heterocysts, sheath, uniseriate or multiseriate trichomes, true or false branching, arrangement of thylakoids, reproduction by akinetes, binary fission, hormogonia, fragmentation, presence/absence of motility etc. Their antiquity, distribution, structural and chemical differentiation, diversity, morphological complexity and large size compared to most other bacteria, makes the cyanobacteria ideal candidates for morphological biomarkers in returned Astromaterials. We have obtained optical (nomarski and phase contrast)/fluorescent (blue and green excitation) microscopy images using an Olympus BX60 compound microscope and Field Emission Scanning Electron Microscopy images and EDAX elemental compositions of living and fossil cyanobacteria. The S-4000 Hitachi Field Emission Scanning Electron Microscope (FESEM) has been used to investigate microfossils in freshly fractured interior surfaces of terrestrial rocks and the cells, hormogonia, sheaths and trichomes of recent filamentous cyanobacteria. We present Fluorescent and FESEM Secondary and Backscattered Electron images and associated EDAX elemental analyses of recent and fossil cyanobacteria, concentrating on representatives of the

  15. Cyanobacteria isolated from the high-intertidal zone: a model for studying the physiological prerequisites for survival in low Earth orbit (United States)

    Olsson-Francis, Karen; Watson, Jonathan S.; Cockell, Charles S.


    Cyanobacteria are capable of surviving the adverse conditions of low Earth orbit (LEO). We have previously demonstrated that Gloeocapsa strain OU_20, Chroococcidiopsis and akinetes of Anabaena cylindrica were able to survive 548 days of exposure to LEO. Motivated by an interest to understand how cyanobacteria can survive in LEO, we studied the strategies that Gloeocapsa strain OU_20 employs to survive in its natural environment, the upper region of the intertidal zone. Here, cyanobacteria are exposed to fluctuations in temperature, UV radiation, desiccation and salinity. We demonstrated that an increase in salinity from 6.5‰ (BG-11 medium) to 35.7‰ (similar to that of seawater), resulted in increased resistance to UV radiation (254 nm), vacuum (0.7×10-3±0.01 kPa) and cold temperatures (-20 °C). Concomitantly, biochemical analyses demonstrated that the amount of fatty acids and mycosporine-like amino acids (a UV absorbing pigment) were higher in the stressed cells. Morphological analysis demonstrated that the electron density and thickness of the mucilaginous sheath were also greater than in the control cells. Yet, the control and stressed cells both formed aggregates. As a result of studying the physiological adaptation of Gloeocapsa strain OU_20 in response to salinity, we postulate that survival in the high-intertidal zone and LEO involves a dense extracellular mucilaginous sheath and the formation of aggregates. We conclude that studying the physiological adaptation of cyanobacteria in the intertidal zone provides insight into understanding survival in LEO.

  16. The smallest known genomes of multicellular and toxic cyanobacteria: comparison, minimal gene sets for linked traits and the evolutionary implications.

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    Karina Stucken

    Full Text Available Cyanobacterial morphology is diverse, ranging from unicellular spheres or rods to multicellular structures such as colonies and filaments. Multicellular species represent an evolutionary strategy to differentiate and compartmentalize certain metabolic functions for reproduction and nitrogen (N(2 fixation into specialized cell types (e.g. akinetes, heterocysts and diazocytes. Only a few filamentous, differentiated cyanobacterial species, with genome sizes over 5 Mb, have been sequenced. We sequenced the genomes of two strains of closely related filamentous cyanobacterial species to yield further insights into the molecular basis of the traits of N(2 fixation, filament formation and cell differentiation. Cylindrospermopsis raciborskii CS-505 is a cylindrospermopsin-producing strain from Australia, whereas Raphidiopsis brookii D9 from Brazil synthesizes neurotoxins associated with paralytic shellfish poisoning (PSP. Despite their different morphology, toxin composition and disjunct geographical distribution, these strains form a monophyletic group. With genome sizes of approximately 3.9 (CS-505 and 3.2 (D9 Mb, these are the smallest genomes described for free-living filamentous cyanobacteria. We observed remarkable gene order conservation (synteny between these genomes despite the difference in repetitive element content, which accounts for most of the genome size difference between them. We show here that the strains share a specific set of 2539 genes with >90% average nucleotide identity. The fact that the CS-505 and D9 genomes are small and streamlined compared to those of other filamentous cyanobacterial species and the lack of the ability for heterocyst formation in strain D9 allowed us to define a core set of genes responsible for each trait in filamentous species. We presume that in strain D9 the ability to form proper heterocysts was secondarily lost together with N(2 fixation capacity. Further comparisons to all available cyanobacterial

  17. The smallest known genomes of multicellular and toxic cyanobacteria: comparison, minimal gene sets for linked traits and the evolutionary implications. (United States)

    Stucken, Karina; John, Uwe; Cembella, Allan; Murillo, Alejandro A; Soto-Liebe, Katia; Fuentes-Valdés, Juan J; Friedel, Maik; Plominsky, Alvaro M; Vásquez, Mónica; Glöckner, Gernot


    Cyanobacterial morphology is diverse, ranging from unicellular spheres or rods to multicellular structures such as colonies and filaments. Multicellular species represent an evolutionary strategy to differentiate and compartmentalize certain metabolic functions for reproduction and nitrogen (N(2)) fixation into specialized cell types (e.g. akinetes, heterocysts and diazocytes). Only a few filamentous, differentiated cyanobacterial species, with genome sizes over 5 Mb, have been sequenced. We sequenced the genomes of two strains of closely related filamentous cyanobacterial species to yield further insights into the molecular basis of the traits of N(2) fixation, filament formation and cell differentiation. Cylindrospermopsis raciborskii CS-505 is a cylindrospermopsin-producing strain from Australia, whereas Raphidiopsis brookii D9 from Brazil synthesizes neurotoxins associated with paralytic shellfish poisoning (PSP). Despite their different morphology, toxin composition and disjunct geographical distribution, these strains form a monophyletic group. With genome sizes of approximately 3.9 (CS-505) and 3.2 (D9) Mb, these are the smallest genomes described for free-living filamentous cyanobacteria. We observed remarkable gene order conservation (synteny) between these genomes despite the difference in repetitive element content, which accounts for most of the genome size difference between them. We show here that the strains share a specific set of 2539 genes with >90% average nucleotide identity. The fact that the CS-505 and D9 genomes are small and streamlined compared to those of other filamentous cyanobacterial species and the lack of the ability for heterocyst formation in strain D9 allowed us to define a core set of genes responsible for each trait in filamentous species. We presume that in strain D9 the ability to form proper heterocysts was secondarily lost together with N(2) fixation capacity. Further comparisons to all available cyanobacterial genomes

  18. Salivary DJ-1 could be an indicator of Parkinson’s disease progression

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    Wen-yan eKang


    Full Text Available Objective: The goal of the current investigation was to explore whether salivary DJ-1 could be a potential biomarker for monitoring disease progression in Parkinson’s disease (PD by evaluating the association between salivary DJ-1 concentrations and nigrostriatal dopaminergic function.Methods: First, in 74 patients with PD and 12 age-matched normal controls, single photon emission computed tomography (SPECT imaging with labeled dopamine transporters (DAT (99mTc-TRODAT-1, which has been used for measuring DAT density in PD was prformed. Then, the DJ-1 level in their saliva was analyzed by quantitative and sensitive Luminex assay and compared to caudate or putamen DAT density. Finally, based on the above, our cross-section study was carried out in 376 research volunteers (285 patients with PD and 91 healthy controls to measure salivary DJ-1 level.Results: From our analysis, we found a correlation between salivary concentration of DJ-1 and putamen nucleus uptake of 99mTc-TRODAT-1 in the PD group. Although salivary DJ-1 levels were not affected by UPDRS scores, gender, age and pharmacotherapy, DJ-1 levels in H&Y 4 stage of PD were higher than those in H&Y 1-3 stage as well as those in healthy controls. Salivary DJ-1 also decreased significantly in mixed type PD patients compared to the tremor-dominant type (TDT and akinetic-rigid dominant type (ARDT PD patients.Conclusions: According to the investigation in a large cohort, we reported for the first time the prognostic potential of the salivary DJ-1 as a biomarker for evaluating nigrostriatal dopaminergic function in PD.

  19. Correlation with neuropsychological assessment and SPM analysis of brain perfusion SPECT in patients with progressive supranuclear palsy

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    Jeong, Young Jin; Kang, Do Young; Park, Kyung Won; Kim, Jae Woo [School of Medicine, Dong-A University, Busan (Korea, Republic of)


    Progressive supranuclear palsy (PSP) is a degenerative condition of unknown aetiology that produces an akinetic-rigid form of parkinsonism characterised by early falls, dementia and abnormalities of extraocular movements. The patterns of decreased regional cerebral blood flow and cognitive impairment in PSP compared with normal control have been insufficiently investigated and a limited number of studies have been performed. We evaluated clinical symptoms, functional neuroimaging study using Tc-99m HMPAO SPECT and neuropsychological profiles in patients with PSP. Eleven patients with PSP diagnosed by the clinical criteria of National Institute of Neurological Disorders and Stroke and the Society for PSP (NINDS-SPSP) (mean age: 70.5{+-}5.6 years, educational period: 4.5{+-}4.7 years) and age-matched 10 healthy control subjects (mean age: 68.1{+-}4.5 years, educational period: 6.5{+-}4.1 years) participated in this study were participated. All patients were given a neurologic examination, brain MRI and cerebral perfusion SPECT using Tc-99m HMPAO. We concomittently evaluated several cognitive profiles using the Seoul Neuropsychological Screening Battery. SPM analysis of the SPECT image showed significant perfusion deficits in the left inferior frontal gyrus, left caudate nucleus, left middle frontal gyrus and cingulate gyrus in the patients with PSP compared with age-matched healthy control (uncorrected p<0.01). On neuropsychological assessment, cognitive deficits on verbal and visual memory, word fluency and frontal executive functions were prominent in most patients with PSP compared with healthy control subjects. Our findings suggest that measurement of regional cerebral blood flow by perfusion SPECT and voxel-based SPM analysis with neuropsychological assessment are useful to understanding the correlation between perfusion deficits and abnormal cognitive profiles in patients with PSP.

  20. Estimulação cerebral profunda no núcleo subtalâmico para doença de Parkinson Deep brain stimulation of subthalamic nucleous in Parkinson's disease

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    José Augusto Nasser


    Full Text Available OBJETIVO: Apresentamos os resultados preliminares da estimulação cerebral profunda crônica no núcleo subtalâmico para os sintomas motores da doença de Parkinson. MÉTODO: 8 pacientes com doença de Parkinson há mais de 5 anos, apresentando tremor incapacitante, oligocinesia e rigidez acentuada com predomínio unilateral, 3 à direita e um à esquerda, refratários �� medicação adequada, foram submetidos ao implante bilateral de eletrodo cerebral profundo permanente subtalâmico, Itrell 2. Estes pacientes foram avaliados segundo as escalas: UPDRS, Schwab e England. RESULTADOS: Os resultados preliminares (6 meses, mostraram melhora significativa dos sinais e sintomas avaliados pelas escalas acima. Não houve complicações com os procedimentos. CONCLUSÃO: Os resultados preliminares indicam que a estimulação subtalâmica é uma boa opção terapêutica, estando de acordo com os resultados da literatura.We present the preliminary results in patients well selected to be implanted by deep brain stimulation (DBS for Parkinsons's disease (PD. METHODS: 8 PD patients with disabled tremor, akinetic/bradikinetic and rigidity, in spite of best therapeutic assay with poor response were referred to surgery. It was implanted DBS Itrell 2. Theses patients were evaluated by the following scales: UPDRS, Schwab and England. RESULTS: The preliminary results in 6 months showed significant improvement of motor performance and it is shown by the scores. There were no complications so far with these procedures. CONCLUSION: The preliminary results indicate that DBS is highly effective, with benefit to those patients and it is according to literature.


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    T. K. Kulua


    Full Text Available Objective: to describe a clinical case of late-stage Parkinson’s disease (PD with nocturnal symptoms and fluctuations and to present successful experience in using the three-component drug Stalevo (levodopa/carbidopa/entacapone as a single dose prior to sleep. Patient V. aged 72 years took medical advice at the Department of Neurology, Russian Medical Academy of Postgraduate Education, for complaints of constrained movements, inability to turn in bed independently, painful muscle cramps, light sleep with nocturnal awakenings, and frequent (5–6 times urinations at night. The first symptoms of the disease appeared 12 years ago when the patient began to notice sluggishness, altered handwriting, and clumsiness during small movements of the right hand. By taking into account symptoms (obvious nocturnal akinesia, overactive bladder, sleep disorders, and cramps and the results of his examination, the patient was diagnosed with the akinetic-rigid form of PD (4 Hoehn-Yarh scores. Therapy was corrected as follows: the dose of amantadine remained the same (300 mg; its last administration is recommended to be at 16:00; the daily dose of levodopa was 700 mg; that of levodopa/benserazide was 550 mg/day (1 tablet × 3/4 tablet × 1 tablet. The evening intake of levodopa was changed for that of the three-component drug Stalevo (levodopa 150 mg/ carbidopa 37.5 mg/entacapone 200 mg. Results. After 3 months, the patient was observed to have significant health improvement: reduced nocturnal and morning akinesia, better gait, a considerably smaller number of nocturnal urinations (less than once per night, regression of painful calf muscle cramps, and improved sleep. Conclusion. The three-component drug Stalevo (levodopa/carbidopa/entacapone is effective in treating late-stage PD. Its use as a single dose before going to bed has led to regressive nocturnal symptoms, better sleep, and lower motor fluctuations. 

  2. Coexistence of protease sensitive and resistant prion protein in 129VV homozygous sporadic Creutzfeldt–Jakob disease: a case report

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    Rodríguez-Martínez Ana B


    Full Text Available Abstract Introduction The coexistence of different molecular types of classical protease-resistant prion protein in the same individual have been described, however, the simultaneous finding of these with the recently described protease-sensitive variant or variably protease-sensitive prionopathy has, to the best of our knowledge, not yet been reported. Case presentation A 74-year-old Caucasian woman showed a sporadic Creutzfeldt–Jakob disease clinical phenotype with reactive depression, followed by cognitive impairment, akinetic-rigid Parkinsonism with pseudobulbar syndrome and gait impairment with motor apraxia, visuospatial disorientation, and evident frontal dysfunction features such as grasping, palmomental reflex and brisk perioral reflexes. She died at age 77. Neuropathological findings showed: spongiform change in the patient’s cerebral cortex, striatum, thalamus and molecular layer of the cerebellum with proteinase K-sensitive synaptic-like, dot-like or target-like prion protein deposition in the cortex, thalamus and striatum; proteinase K-resistant prion protein in the same regions; and elongated plaque-like proteinase K-resistant prion protein in the molecular layer of the cerebellum. Molecular analysis of prion protein after proteinase K digestion revealed decreased signal intensity in immunoblot, a ladder-like protein pattern, and a 71% reduction of PrPSc signal relative to non-digested material. Her cerebellum showed a 2A prion protein type largely resistant to proteinase K. Genotype of polymorphism at codon 129 was valine homozygous. Conclusion Molecular typing of prion protein along with clinical and neuropathological data revealed, to the best of our knowledge, the first case of the coexistence of different protease-sensitive prion proteins in the same patient in a rare case that did not fulfill the current clinical diagnostic criteria for either probable or possible sporadic Creutzfeldt–Jakob disease. This highlights the

  3. New Perspective on Parkinsonism in Frontotemporal Lobar Degeneration

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    Hee Kyung Park


    Full Text Available Frontotemporal dementia (FTD is the second most common type of presenile dementia. Three clinical prototypes have been defined; behavioral variant FTD, semantic dementia, and progressive nonfluent aphasia. Progressive supranuclear palsy, corticobasal degeneration, and motor neuron disease may possess clinical and pathological characteristics that overlap with FTD, and it is possible that they may all belong to the same clinicopathological spectrum. Frontotemporal lobar degeneration (FTLD is a clinicopathological syndrome that encompasses a heterogenous group of neurodegenerative disorders. Owing to the advancement in the field of molecular genetics, diagnostic imaging, and pathology, FTLD has been the focus of great interest. Nevertheless, parkinsonism in FTLD has received relatively less attention. Parkinsonism is found in approximately 20–30% of patients in FTLD. Furthermore, parkinsonism can be seen in all FTLD subtypes, and some patients with familial and sporadic FTLD can present with prominent parkinsonism. Therefore, there is a need to understand parkinsonism in FTLD in order to obtain a better understanding of the disease. With regard to the clinical characteristics, the akinetic rigid type of parkinsonism has predominantly been described. Parkinsonism is frequently observed in familial FTD, more specifically, in FTD with parkinsonism linked to chromosome 17q (FTDP-17. The genes associated with parkinsonism are microtubule associated protein tau (MAPT, progranulin (GRN or PGRN, and chromosome 9 open reading frame 72 (C9ORF72 repeat expansion. The neural substrate of parkinsonism remains to be unveiled. Dopamine transporter (DAT imaging revealed decreased uptake of DAT, and imaging findings indicated atrophic changes of the basal ganglia. Parkinsonism can be an important feature in FTLD and, therefore, increased attention is needed on the subject.

  4. Parkinsonian syndrome in familial frontotemporal dementia. (United States)

    Siuda, Joanna; Fujioka, Shinsuke; Wszolek, Zbigniew K


    Parkinsonism in frontotemporal dementia (FTD) was first described in families with mutations in the microtubule-associated protein tau (MAPT) and progranulin (PRGN) genes. Since then, mutations in several other genes have been identified for FTD with parkinsonism, including chromosome 9 open reading frame 72 (C9ORF72), chromatin modifying protein 2B (CHMP2B), valosin-containing protein (VCP), fused in sarcoma (FUS) and transactive DNA-binding protein (TARDBP). The clinical presentation of patients with familial forms of FTD with parkinsonism is highly variable. The parkinsonism seen in FTD patients is usually characterized by akinetic-rigid syndrome and is mostly associated with the behavioral variant of FTD (bvFTD); however, some cases may present with classical Parkinson's disease. In other cases, atypical parkinsonism resembling progressive supranuclear palsy (PSP) or corticobasal syndrome (CBS) has also been described. Although rare, parkinsonism in FTD may coexist with motor neuron disease. Structural neuroimaging, which is crucial for the diagnosis of FTD, shows characteristic patterns of brain atrophy associated with specific mutations. Structural neuroimaging is not helpful in distinguishing among patients with parkinsonian features. Furthermore, dopaminergic imaging that shows nigrostriatal neurodegeneration in FTD with parkinsonism cannot discriminate parkinsonian syndromes that arise from different mutations. Generally, parkinsonism in FTD is levodopa unresponsive, but there have been cases where a temporary benefit has been reported, so dopaminergic treatment is worth trying, especially, when motor and non-motor manifestations can cause significant problems with daily functioning. In this review, we present an update on the clinical and genetic correlations of FTD with parkinsonism.

  5. Morphology and Elemental Composition of Recent and Fossil Cyanobacteria (United States)

    SaintAmand, Ann; Hoover, Richard B.; Jerman, Gregory; Rozanov, Alexei Yu.


    Cyanobacteria (cyanophyta, cyanoprokaryota, and blue-green algae) are an ancient, diverse and abundant group of photosynthetic oxygenic microorganisms. Together with other bacteria and archaea, the cyanobacteria have been the dominant life forms on Earth for over 3.5 billion years. Cyanobacteria occur in some of our planets most extreme environments - hot springs and geysers, hypersaline and alkaline lakes, hot and cold deserts, and the polar ice caps. They occur in a wide variety of morphologies. Unlike archaea and other bacteria, which are typically classified in pure culture by their physiological, biochemical and phylogenetic properties, the cyanobacteria have historically been classified based upon their size and morphological characteristics. These include the presence or absence of heterocysts, sheath, uniseriate or multiseriate trichomes, true or false branching, arrangement of thylakoids, reproduction by akinetes, binary fission, hormogonia, fragmentation, presence/absence of motility etc. Their antiquity, distribution, structural and chemical differentiation, diversity, morphological complexity and large size compared to most other bacteria, makes the cyanobacteria ideal candidates for morphological biomarkers in returned Astromaterials. We have obtained optical (nomarski and phase contrast)/fluorescent (blue and green excitation) microscopy images using an Olympus BX60 compound microscope and Field Emission Scanning Electron Microscopy images and EDAX elemental compositions of living and fossil cyanobacteria. The S-4000 Hitachi Field Emission Scanning Electron Microscope (FESEM) has been used to investigate microfossils in freshly fractured interior surfaces of terrestrial rocks and the cells, hormogonia, sheaths and trichomes of recent filamentous cyanobacteria. We present Fluorescent and FESEM Secondary and Backscattered Electron images and associated EDAX elemental analyses of recent and fossil cyanobacteria, concentrating on representatives of the

  6. Impaired sense of smell in a Drosophila Parkinson's model.

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    Simone Poddighe

    Full Text Available Parkinson's disease (PD is one of the most common neurodegenerative disease characterized by the clinical triad: tremor, akinesia and rigidity. Several studies have suggested that PD patients show disturbances in olfaction at the earliest onset of the disease. The fruit fly Drosophila melanogaster is becoming a powerful model organism to study neurodegenerative diseases. We sought to use this system to explore olfactory dysfunction, if any, in PINK1 mutants, which is a model for PD. PINK1 mutants display many important diagnostic symptoms of the disease such as akinetic motor behavior. In the present study, we describe for the first time, to the best of our knowledge, neurophysiological and neuroanatomical results concerning the olfactory function in PINK1 mutant flies. Electroantennograms were recorded in response to synthetic and natural volatiles (essential oils from groups of PINK1 mutant adults at three different time points in their life cycle: one from 3-5 day-old flies, from 15-20 and from 27-30 days. The results obtained were compared with the same age-groups of wild type flies. We found that mutant adults showed a decrease in the olfactory response to 1-hexanol, α-pinene and essential oil volatiles. This olfactory response in mutant adults decreased even more as the flies aged. Immunohistological analysis of the antennal lobes in these mutants revealed structural abnormalities, especially in the expression of Bruchpilot protein, a marker for synaptic active zones. The combination of electrophysiological and morphological results suggests that the altered synaptic organization may be due to a neurodegenerative process. Our results indicate that this model can be used as a tool for understanding PD pathogensis and pathophysiology. These results help to explore the potential of using olfaction as a means of monitoring PD progression and developing new treatments.

  7. Subthalamic local field potentials in Parkinson's disease and isolated dystonia: An evaluation of potential biomarkers. (United States)

    Wang, Doris D; de Hemptinne, Coralie; Miocinovic, Svjetlana; Qasim, Salman E; Miller, Andrew M; Ostrem, Jill L; Galifianakis, Nicholas B; San Luciano, Marta; Starr, Philip A


    Local field potentials (LFP) recorded from the subthalamic nucleus in patients with Parkinson's disease (PD) demonstrate prominent oscillations in the beta (13-30 Hz) frequency range, and reduction of beta band spectral power by levodopa and deep brain stimulation (DBS) is correlated with motor symptom improvement. Several features of beta activity have been theorized to be specific biomarkers of the parkinsonian state, though these have rarely been studied in non-parkinsonian conditions. To compare resting state LFP features in PD and isolated dystonia and evaluate disease-specific biomarkers, we recorded subthalamic LFPs from 28 akinetic-rigid PD and 12 isolated dystonia patients during awake DBS implantation. Spectral power and phase-amplitude coupling characteristics were analyzed. In 26/28 PD and 11/12 isolated dystonia patients, the LFP power spectrum had a peak in the beta frequency range, with similar amplitudes between groups. Resting state power did not differ between groups in the theta (5-8 Hz), alpha (8-12 Hz), beta (13-30 Hz), broadband gamma (50-200 Hz), or high frequency oscillation (HFO, 250-350 Hz) bands. Analysis of phase-amplitude coupling between low frequency phase and HFO amplitude revealed significant interactions in 19/28 PD and 6/12 dystonia recordings without significant differences in maximal coupling or preferred phase. Two features of subthalamic LFPs that have been proposed as specific parkinsonian biomarkers, beta power and coupling of beta phase to HFO amplitude, were also present in isolated dystonia, including focal dystonias. This casts doubt on the utility of these metrics as disease-specific diagnostic biomarkers.

  8. Xenon contrast CT-CBF measurements in parkinsonism and normal aging. (United States)

    Tachibana, H; Meyer, J S; Kitagawa, Y; Tanahashi, N; Kandula, P; Rogers, R L


    Local cerebral blood flow (LCBF) and local tissue:blood partition, coefficient (L lambda) values were measured during CT scanning while patients with different types of Parkinson's syndrome (N = 14) inhaled a contrast mixture of 35-37 per cent stable xenon gas in oxygen. Single-compartment analysis fitted to infinity was used to calculate L lambda and LCBF values. Results were compared with results from normal age-matched volunteers (N = 24). Mean hemispheric (p less than 0.05) and subcortical (p less than 0.05) gray matter LCBF values were reduced in idiopathic Parkinson's disease (N = 11), compared to values from age-matched normals. Regionally, LCBF reductions included frontal (p less than 0.001), parietal cortex (p less than 0.05), caudate (p less than 0.05), lentiform nuclei (p less than 0.001) and thalamus (p less than 0.05) reductions. L lambda values were normal. Unilateral tremor and/or rigidity correlated directly with reduced LCBF in contralateral lentiform (p less than 0.01) and caudate (p less than 0.01) nuclei. In postencephalitic Parkinsonism (N = 1) LCBF reductions were diffuse, with normal L lambda values. In the akinetic form of Parkinsonism (N = 1) associated with lacunar infarcts, LCBF and L lambda reductions were patchy. In Parkinsonism following carbon monoxide poisoning (N = 1), LCBF values of gray and white matter were diffusely reduced and L lambda values were reduced in both pallidal regions. When dementia was present together with Parkinsonism (N = 3), LCBF reductions were more diffuse and severe. Dopaminergic deficiency correlated directly with reduced LCBF values, reflecting the severity of Parkinsonism.

  9. A transitional snake from the Late Cretaceous period of North America. (United States)

    Longrich, Nicholas R; Bhullar, Bhart-Anjan S; Gauthier, Jacques A


    Snakes are the most diverse group of lizards, but their origins and early evolution remain poorly understood owing to a lack of transitional forms. Several major issues remain outstanding, such as whether snakes originated in a marine or terrestrial environment and how their unique feeding mechanism evolved. The Cretaceous Coniophis precedens was among the first Mesozoic snakes discovered, but until now only an isolated vertebra has been described and it has therefore been overlooked in discussions of snake evolution. Here we report on previously undescribed material from this ancient snake, including the maxilla, dentary and additional vertebrae. Coniophis is not an anilioid as previously thought a revised phylogenetic analysis of Ophidia shows that it instead represents the most primitive known snake. Accordingly, its morphology and ecology are critical to understanding snake evolution. Coniophis occurs in a continental floodplain environment, consistent with a terrestrial rather than a marine origin; furthermore, its small size and reduced neural spines indicate fossorial habits, suggesting that snakes evolved from burrowing lizards. The skull is intermediate between that of lizards and snakes. Hooked teeth and an intramandibular joint indicate that Coniophis fed on relatively large, soft-bodied prey. However, the maxilla is firmly united with the skull, indicating an akinetic rostrum. Coniophis therefore represents a transitional snake, combining a snake-like body and a lizard-like head. Subsequent to the evolution of a serpentine body and carnivory, snakes evolved a highly specialized, kinetic skull, which was followed by a major adaptive radiation in the Early Cretaceous period. This pattern suggests that the kinetic skull was a key innovation that permitted the diversification of snakes.

  10. [Neuroleptic malignant syndrome associated with the syndrome of inappropriate secretion of antidiuretic hormone]. (United States)

    Yamaguchi, K; Takamoto, K; Yagi, K; Tanabe, H


    We reported a case of neuroleptic malignant syndrome (NMS) associated with the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). A 71-year-old woman, who had been diagnosed as hypertension and multiple cerebral infarction, was given sulpiride 150 mg daily for depressive state. Three days after started sulpiride, she developed fever, sweating, difficulty of movement and was admitted to the hospital. The white blood cell count rose to 16,300/mm3 and serum creatine kinase (CK) to 3,063 IU/L. Two days later CK rose to 20,050 IU/L regardless of stopping the drug, so she was transferred to our hospital for further investigation. On admission, it was the 6th day from the onset, she was mute and akinetic accompanied by muscle pain and rigidity in extremities. Serum CK was 1,831 IU/L, Na 122 mEq/L, osmolality 244 mOsm/kg, plasma antidiuretic hormone (ADH) level 6.5 pg/ml and urine Na was 101 mEq/L, osmolality 467 mOsm/kg. Renal and adrenal functions, plasma renin activity were normal. From the history, course and these data, diagnosis of NMS associated with SIADH was made. Intravenous sodium (130-200 mEq/day) and fluids (1,000-1,200 ml/day) were carefully infused. She became active, muscle pain disappeared and rigidity, akinesia decreased. CK, serum Na and osmolality gradually improved to normal. About the transient increase in ADH secretion, we considered that hypothalamic disturbance in NMS might induce leakage of stored ADH from neuroendocrine neurons in it.

  11. Riluzole treatment, survival and diagnostic criteria in Parkinson plus disorders: the NNIPPS study. (United States)

    Bensimon, Gilbert; Ludolph, Albert; Agid, Yves; Vidailhet, Marie; Payan, Christine; Leigh, P Nigel


    Parkinson plus diseases, comprising mainly progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) are rare neurodegenerative conditions. We designed a double-blind randomized placebo-controlled trial of riluzole as a potential disease-modifying agent in Parkinson plus disorders (NNIPPS: Neuroprotection and Natural History in Parkinson Plus Syndromes). We analysed the accuracy of our clinical diagnostic criteria, and studied prognostic factors for survival. Patients with an akinetic-rigid syndrome diagnosed as having PSP or MSA according to modified consensus diagnostic criteria were considered for inclusion. The psychometric validity (convergent and predictive) of the NNIPPS diagnostic criteria were tested prospectively by clinical and pathological assessments. The study was powered to detect a 40% decrease in relative risk of death within PSP or MSA strata. Patients were randomized to riluzole or matched placebo daily and followed up to 36 months. The primary endpoint was survival. Secondary efficacy outcomes were rates of disease progression assessed by functional measures. A total of 767 patients were randomized and 760 qualified for the Intent to Treat (ITT) analysis, stratified at entry as PSP (362 patients) or MSA (398 patients). Median follow-up was 1095 days (range 249-1095). During the study, 342 patients died and 112 brains were examined for pathology. NNIPPS diagnostic criteria showed for both PSP and MSA excellent convergent validity with the investigators' assessment of diagnostic probability (point-biserial correlation: MSA r(pb) = 0.93, P diagnostic criteria were consistent and valid. They can be used to distinguish between PSP and MSA with high accuracy, and should facilitate research into these conditions relatively early in their evolution.

  12. Bee Venom Alleviates Motor Deficits and Modulates the Transfer of Cortical Information through the Basal Ganglia in Rat Models of Parkinson’s Disease (United States)

    Maurice, Nicolas; Deltheil, Thierry; Melon, Christophe; Degos, Bertrand; Mourre, Christiane


    Recent evidence points to a neuroprotective action of bee venom on nigral dopamine neurons in animal models of Parkinson’s disease (PD). Here we examined whether bee venom also displays a symptomatic action by acting on the pathological functioning of the basal ganglia in rat PD models. Bee venom effects were assessed by combining motor behavior analyses and in vivo electrophysiological recordings in the substantia nigra pars reticulata (SNr, basal ganglia output structure) in pharmacological (neuroleptic treatment) and lesional (unilateral intranigral 6-hydroxydopamine injection) PD models. In the hemi-parkinsonian 6-hydroxydopamine lesion model, subchronic bee venom treatment significantly alleviates contralateral forelimb akinesia and apomorphine-induced rotations. Moreover, a single injection of bee venom reverses haloperidol-induced catalepsy, a pharmacological model reminiscent of parkinsonian akinetic deficit. This effect is mimicked by apamin, a blocker of small conductance Ca2+-activated K+ (SK) channels, and blocked by CyPPA, a positive modulator of these channels, suggesting the involvement of SK channels in the bee venom antiparkinsonian action. In vivo electrophysiological recordings in the substantia nigra pars reticulata (basal ganglia output structure) showed no significant effect of BV on the mean neuronal discharge frequency or pathological bursting activity. In contrast, analyses of the neuronal responses evoked by motor cortex stimulation show that bee venom reverses the 6-OHDA- and neuroleptic-induced biases in the influence exerted by the direct inhibitory and indirect excitatory striatonigral circuits. These data provide the first evidence for a beneficial action of bee venom on the pathological functioning of the cortico-basal ganglia circuits underlying motor PD symptoms with potential relevance to the symptomatic treatment of this disease. PMID:26571268

  13. Medulloblastoma in children: Prognostic factors and predictors of outcome

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    Girish Menon


    Full Text Available Objective: To determine the relative contributions of clinical, radiological and histopatholgical predictors of survival in children with medulloblastoma (MB and to compare it with their adult counterparts. Materials and Methods: Retrospective case record analyses of 79 children (< 16 y operated after Jan. 1990, who have completed at least 5 y of follow-up. The following variables were assessed by bivariate analysis: age, CT scan location of the lesion, brainstem invasion, extent of excision, histological subtype. Statistical analysis was performed using Chi-square test, Fischers test and Student′s t test. Results: Near-total to total excision could be achieved in 59 (74.6% cases. Twenty-three patients (29.11% required CSF diversion procedures. Histopathology revealed features of classical medulloblastoma in 63.2%, thermoplastic variant in 11% and glial differentiation in 25.3% of cases. Postoperative mutism was seen in 14 (17.72% patients. All patients received adjuvant therapy. On follow-up, 34 patients were found to have posterior fossa recurrence and four patients were re-operated. An additional 17% of patients were found to have either spinal or supratentorial metastasis on follow-up. The overall 5-year recurrence-free survival rate was 19 (24.05%. Mortality was recorded in 23 patients and nearly 29 patients who were severely disabled on follow-up were referred to terminal care centres. Conclusion: In spite of recent advances in management, children with medulloblastoma still carry a poor prognosis. We observed poor outcome in children below 7 y of age. Vermian location had a better outcome in adults but not in children. Desmoplastic variant was observed to be a significant prognostic factor in paediatric, group while brain stem invasion carried poor prognosis for both.

  14. Extending parent-child interaction therapy for early childhood internalizing problems: new advances for an overlooked population. (United States)

    Carpenter, Aubrey L; Puliafico, Anthony C; Kurtz, Steven M S; Pincus, Donna B; Comer, Jonathan S


    Although efficacious psychological treatments for internalizing disorders are now well established for school-aged children, until recently there have regrettably been limited empirical efforts to clarify indicated psychological intervention methods for the treatment of mood and anxiety disorders presenting in early childhood. Young children lack many of the developmental capacities required to effectively participate in established treatments for mood and anxiety problems presenting in older children, making simple downward extensions of these treatments for the management of preschool internalizing problems misguided. In recent years, a number of research groups have successfully adapted and modified parent-child interaction therapy (PCIT), originally developed to treat externalizing problems in young children, to treat various early internalizing problems with a set of neighboring protocols. As in traditional PCIT, these extensions target child symptoms by directly reshaping parent-child interaction patterns associated with the maintenance of symptoms. The present review outlines this emerging set of novel PCIT adaptations and modifications for mood and anxiety problems in young children and reviews preliminary evidence supporting their use. Specifically, we cover (a) PCIT for early separation anxiety disorder; (b) the PCIT-CALM (Coaching Approach behavior and Leading by Modeling) Program for the full range of early anxiety disorders; (c) the group Turtle Program for behavioral inhibition; and (d) the PCIT-ED (Emotional Development) Program for preschool depression. In addition, emerging PCIT-related protocols in need of empirical attention--such as the PCIT-SM (selective mutism) Program for young children with SM--are also considered. Implications of these protocols are discussed with regard to their unique potential to address the clinical needs of young children with internalizing problems. Obstacles to broad dissemination are addressed, and we consider

  15. Two different manifestations of locked-in syndrome. (United States)

    Golubović, Vesna; Muhvić, Damir; Golubović, Snjezana; Juretić, Mirna; Tokmadzić, Vlatka Sotosek


    Locked-in syndrome (LIS) is an entity that usually occur a consequence of the lesion of ventral part of pons. Etiology of locked-in syndrome can be vascular and nonvascular origin. Locked-in syndrome usually occurs as a consequence of thrombosis of intermedial segment of basilar artery that induces bilateral infarction of the ventrobasal part of the pons. Additionally, LIS can be caused by trauma which often leads to posttraumatic thrombosis of basilar artery. The incidence of locked-in syndrome is still unknown. The basic clinical features of locked-in syndrome are: quadriplegia (a consequence of disruption of corticospinal pathways located in ventral part of pons), different stages of paralysis of mimic musculature, paralysis of pharynx, tongue and palate with mutism and anarthria. The patient can not move, but is conscious and can communicate only by eye movements. Two patients with locked-in syndrome were present in this article. In the first case, the patient had classic locked-in syndrome that was first described by Plum and Posner. Other patient had incomplete form of locket-in syndrome which was first described by Bauer. In these two patients locked-in syndrome occurred as a consequence of trauma. In the first patient locked-in syndrome was caused by direct contusion of ventral part of pons while in other patient locked-in syndrome was a consequence of posttraumatic thrombosis of vertebrobasilar artery. The introduction of anticoagulant therapy, besides the other measures of intensive therapy, has shown complete justification in the second patient. The gradual partial recovery of neurologic deficit has developed in the second patient without any additional complications.

  16. Síndrome de Cotard asociado a Trastorno Depresivo Mayor con síntomas catatónicos. Informe de caso / Cotard Syndrome Associated to Major Depressive Disorder with Catatonic Symptoms. Case report / Síndrome de Cotard associada ao transtorno depressivo maior com sintomas catatônicos. Relato de caso

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    Daniel Mauricio Torrado-Arenas


    Full Text Available Introduction: Catatonia is a neuropsychiatric syndrome with abnormal postures, mutism and stupor. Colombia has a prevalence of 11.4% of psychiatric patients. Objective: To discuss the clinical curse of a 34-year-old woman with major depressive disorder that presents to emergency department with nihilistic delirium and catatonic symptoms. Case presentation: A young woman with history of unipolar major depression with psychotic features was hospitalized nine months ago. She was medicated with a pharmacological treatment she did not remember. At admission, the patient had three days of bizarre behavior, mutism and negativism. Paraclinics and brain computer tomography did not report any abnormality or changes. Treatment began with benzodiazepine, which achieved full remission of catatonic symptoms. After this, she developed anhedonia, sadness and nihilistic delusions and was considered as a relapse of a previous depressive episode from nine months ago, associated with Cotard’s syndrome. Sertraline was added with gradual increase to 100mg and 5mg of olanzapine, getting a complete remission of psychotic and mood symptoms. Discussion: Affective disorders are most common cause of catatonia. There has already been a history of similar reports, but in few times these three entities were associated; this is the first case reported in Hospital Universitario de Santander, with informed consent. Conclusions: It is unusual for a depressed patient to present denial delusions and catatonic symptoms simultaneously; therefore this case is unusual and may contribute to literature. The catatonic symptoms make it difficult to explore other mental spheres, though they may be secondary to a medical condition, therefore, it is essential to dismiss organic pathologies and give initial treatment, so we can discover the underlying etiopsychopathology. [Torrado-Arenas DM, Santos-Gutiérrez KE, Ruiz-Higuera SM, Zabala-Arias LM, Niño-García JA. Síndrome de Cotard

  17. World War I psychoneuroses: hysteria goes to war. (United States)

    Tatu, Laurent; Bogousslavsky, Julien


    During the First World War, military physicians from the belligerent countries were faced with soldiers suffering from psychotrauma with often unheard of clinical signs, such as camptocormia. These varied clinical presentations took the form of abnormal movements, deaf-mutism, mental confusion, and delusional disorders. In Anglo-Saxon countries, the term 'shell shock' was used to define these disorders. The debate on whether the war was responsible for these disorders divided mobilized neuropsychiatrists. In psychological theories, war is seen as the principal causal factor. In hystero-pithiatism, developed by Joseph Babinski (1857-1932), trauma was not directly caused by the war. It was rather due to the unwillingness of the soldier to take part in the war. Permanent suspicion of malingering resulted in the establishment of a wide range of medical experiments. Many doctors used aggressive treatment methods to force the soldiers exhibiting war neuroses to return to the front as quickly as possible. Medicomilitary collusion ensued. Electrotherapy became the basis of repressive psychotherapy, such as 'torpillage', which was developed by Clovis Vincent (1879-1947), or psychofaradism, which was established by Gustave Roussy (1874-1948). Some soldiers refused such treatments, considering them a form of torture, and were brought before courts-martial. Famous cases, such as that of Baptiste Deschamps (1881-1953), raised the question of the rights of the wounded. Soldiers suffering from psychotrauma, ignored and regarded as malingerers or deserters, were sentenced to death by the courts-martial. Trials of soldiers or doctors were also held in Germany and Austria. After the war, psychoneurotics long haunted asylums and rehabilitation centers. Abuses related to the treatment of the Great War psychoneuroses nevertheless significantly changed medical concepts, leading to the modern definition of 'posttraumatic stress disorder'.

  18. Longitudinal MRI assessment: the identification of relevant features in the development of Posterior Fossa Syndrome in children (United States)

    Spiteri, M.; Lewis, E.; Windridge, D.; Avula, S.


    Up to 25% of children who undergo brain tumour resection surgery in the posterior fossa develop posterior fossa syndrome (PFS). This syndrome is characterised by mutism and disturbance in speech. Our hypothesis is that there is a correlation between PFS and the occurrence of hypertrophic olivary degeneration (HOD) in lobes within the posterior fossa, known as the inferior olivary nuclei (ION). HOD is exhibited as an increase in size and intensity of the ION on an MR image. Intra-operative MRI (IoMRI) is used during surgical procedures at the Alder Hey Children's Hospital, Liver- pool, England, in the treatment of Posterior Fossa tumours and allows visualisation of the brain during surgery. The final MR scan on the IoMRI allows early assessment of the ION immediately after the surgical procedure. The longitudinal MRI data of 28 patients was analysed in a collaborative study with Alder Hey Children's Hospital, in order to identify the most relevant imaging features that relate to the development of PFS, specifically related to HOD. A semi-automated segmentation process was carried out to delineate the ION on each MRI. Feature selection techniques were used to identify the most relevant features amongst the MRI data, demographics and clinical data provided by the hospital. A support vector machine (SVM) was used to analyse the discriminative ability of the selected features. The results indicate the presence of HOD as the most efficient feature that correlates with the development of PFS, followed by the change in intensity and size of the ION and whether HOD occurred bilaterally or unilaterally.

  19. Neuroleptic intolerance in patients with anti-NMDAR encephalitis (United States)

    Lejuste, Florian; Thomas, Laure; Picard, Géraldine; Desestret, Virginie; Ducray, François; Rogemond, Veronique; Psimaras, Dimitri; Antoine, Jean-Christophe; Delattre, Jean-Yves; Groc, Laurent; Leboyer, Marion


    Objective: To precisely describe the initial psychiatric presentation of patients with anti-NMDA receptor (NMDAR) antibodies encephalitis (anti-NMDAR encephalitis) to identify potential clues enhancing its early diagnosis. Methods: We retrospectively studied the French Reference Centre medical records of every adult patient with anti-NMDAR encephalitis to specify the patients' initial psychiatric symptoms leading to hospitalization in a psychiatric department and the reasons underlying the diagnosis of anti-NMDAR encephalitis. Results: The medical records of 111 adult patients were reviewed. Psychiatric features were the initial presentation in 65 patients (59%). Among them, several psychiatric manifestations were observed, including visual and auditory hallucinations (n = 26, 40%), depression (n = 15, 23%), mania (n = 5, 8%), acute schizoaffective episode (n = 15, 23%), and eating disorder or addiction (n = 4; 6%). Forty-five patients (40% of total cohort) were first hospitalized in a psychiatric institution (91% women), with a median duration of stay of 9 days (range 0.25–239 days). Among them, 24 patients (53%) had associated discreet neurologic signs at the first evaluation, while 17 additional patients (38%) developed neurologic signs within a few days. Twenty-one patients (47%) were transferred to a medical unit for a suspicion of antipsychotic intolerance characterized by high temperature, muscle rigidity, mutism or coma, and biological results suggesting rhabdomyolysis. Conclusions: Several psychiatric presentations were observed in patients with anti-NMDAR encephalitis, although none was specific; however, patients, mostly women, also had discreet neurologic signs that should be carefully assessed as well as signs of antipsychotic intolerance that should raise suspicion for anti-NMDAR encephalitis. PMID:27606355

  20. Microsurgical management of pediatric ependymomas of the fourth ventricle via the trans-cerebellomedullary fissure approach: A review of 26 cases (United States)



    In the present study, the microsurgical management of 26 ependymomas of the fourth ventricle in children via the trans-cerebellomedullary fissure (CMF) approach was reviewed and evaluated. Clinical data were obtained from 26 ependymomas of the fourth ventricle treated with microsurgery using the trans-CMF approach from March 2006 to September 2010 at the Department of Neurosurgery of The First Affiliated Hospital of China Medical University (Shenyang, China). These data were collected and analyzed. Suboccipital median posterior fossa craniotomy and trans-CMF approach were performed in all cases for the microsurgical removal of the tumors. An additional incision was performed in the inferior medullary velum of 5 patients, in order to obtain adequate exposure of the tumors. As a result, all tumors were well exposed during surgery. Gross total resection (GTR) was achieved in 22 cases, near total resection (NTR) in 3 cases and subtotal resection (STR) in 1 case. All excised tumors were pathologically confirmed. No mortality occurred intraoperatively, and no patient presented with mutism or any other surgery-related complications. One patient suffered from postoperative hydrocephalus and received ventriculoperitoneal shunting, which relieved the symptoms. Over the 3.0–7.5-year follow-up period (mean, 4.8 years), tumor relapse occurred in 1 case with GTR, 2 cases with NTR and 1 case with STR. In total, 3 patients succumbed to tumor relapse and 4 were lost to follow-up. According to the literature and the clinical experience of the present authors, the trans-CMF approach provides safe and sufficient access to the fourth ventricle without the requirement of an incision in the inferior vermis. This approach prevents damage to the normal cerebellum and improves the surgical outcome. Tumor removal, restoration of cerebrospinal fluid circulation and preservation of brainstem function are factors that should be taken into consideration during surgery. For patients with

  1. DTI fiber tractography of cerebro-cerebellar pathways and clinical evaluation of ataxia in childhood posterior fossa tumor survivors. (United States)

    Oh, Myung Eun; Driever, Pablo Hernáiz; Khajuria, Rajiv K; Rueckriegel, Stefan Mark; Koustenis, Elisabeth; Bruhn, Harald; Thomale, Ulrich-Wilhelm


    Pediatric posterior fossa (PF) tumor survivors experience long-term motor deficits. Specific cerebrocerebellar connections may be involved in incidence and severity of motor dysfunction. We examined the relationship between long-term ataxia as well as fine motor function and alteration of differential cerebellar efferent and afferent pathways using diffusion tensor imaging (DTI) and tractography. DTI-based tractography was performed in 19 patients (10 pilocytic astrocytoma (PA) and 9 medulloblastoma patients (MB)) and 20 healthy peers. Efferent Cerebello-Thalamo-Cerebral (CTC) and afferent Cerebro-Ponto-Cerebellar (CPC) tracts were reconstructed and analyzed concerning fractional anisotropy (FA) and volumetric measurements. Clinical outcome was assessed with the International Cooperative Ataxia Rating Scale (ICARS). Kinematic parameters of fine motor function (speed, automation, variability, and pressure) were obtained by employing a digitizing graphic tablet. ICARS scores were significantly higher in MB patients than in PA patients. Poorer ICARS scores and impaired fine motor function correlated significantly with volume loss of CTC pathway in MB patients, but not in PA patients. Patients with pediatric post-operative cerebellar mutism syndrome showed higher loss of CTC pathway volume and were more atactic. CPC pathway volume was significantly reduced in PA patients, but not in MB patients. Neither relationship was observed between the CPC pathway and ICARS or fine motor function. There was no group difference of FA values between the patients and healthy peers. Reduced CTC pathway volumes in our cohorts were associated with severity of long-term ataxia and impaired fine motor function in survivors of MBs. We suggest that the CTC pathway seems to play a role in extent of ataxia and fine motor dysfunction after childhood cerebellar tumor treatment. DTI may be a useful tool to identify relevant structures of the CTC pathway and possibly avoid surgically induced long


    Directory of Open Access Journals (Sweden)

    N. V. Fedorova


    Full Text Available Objective – a description of clinical cases of impulsive­compulsive disorders in Parkinson,s disease. The first clinical case. Patient N., 75 years old, suffering for 15 years from Parkinson,s disease, akinetic­rigid form of the disease, stage 4 by Hyun–Yar. Since 2009, he received levodopa/carbidopa 250/25 mg 5 times/day (daily dose of 1250 mg of levodopa; pramipexole 3.5 mg per day (daily dose 3.5 mg, amantadine sulfate 100 mg 5 times/day (daily dose 500 mg. While taking antiparkinsonian drugs the patient developed behavioral disorders such as dopamine disregulatory syndrome combined with punding, hypersexuality and compulsive shopping accompanied by visual hallucinations. The total equivalent dose of levodopa was 1600 mg per day. The second clinical case. Patient R., 52 years old, suffers from Parkinson,s disease about 5 years, a mixed form. She complained of slowness of movement, tremor in her left hand, sleep disturbances, poor mood. The clinic was appointed piribedil 50 mg 3 times per day. Despite the fact that the patient took only one of dopaminergic drugs in a therapeutic daily dose, she developed impulsive­compulsive disorder as hyper­ sexuality, compulsive shopping and binge eating. Results. In the first clinical case for correction of behavioral disorders in patients with Parkinson,s disease levodopa/carbidopa dose was reduced to 750 mg per day (3/4 Tab. 4 times a day; added to levodopa/benserazide dispersible 100 mg morning and levodopa/benserazide 100 mg before sleep (total dose of levodopa of 950 mg per day. Amantadine sulfate and pramipexole were canceled. It was added to the therapy of atypical neuroleptic clozapine dose 6,25 mg overnight. After 3 months marked improvement, regressed visual hallucina­ tions, improved family relationships, background mood became more stable. The patient continue to sing karaoke, but this hobby has be­ come less intrusive. In the second clinical event correction impulsive

  3. [Alternative surgical options to heart transplantation]. (United States)

    Dreyfus, G


    Cardiac transplantation is the treatment of reference for refractory cardiac failure but the limited number of donors, the complications inherent to transplantation and the relative and absolute contra-indications has made it necessary to find alternative surgical solutions. The detection of myocardial viability by Thallium scintigraphy, Dobutamine echocardiography and/or position emission tomography in coronary disease, allows identification of zones which are capable of recovering contractile function after revascularisation. The authors report the results of a series of 91 operated patients with a 10 year follow-up having a 72% 5 year actuarial survival and improved ejection fraction. The other alternative which may improve symptoms and prognosis in patients with severe ischaemic heart disease with left ventricular dysfunction is apical remodelling or Dor's procedure. The results of a haemodynamic study at 1 year of 171 patients clearly show a functional improvement and an increase of the ejection fraction. The advantage of this method is that it can be used in patients with dyskinetic and akinetic plaques resulting from antero-septo-apical infarction. Finally, even if mitral regurgitation is relatively uncommon in chronic ischaemic heart disease, a simple procedure (annuloplasty) is often sufficient to correct the mitral regurgitation and reduce the afterload of a failing ventricle. On the other hand, in dilated cardiomyopathy, two new options have been developed; one, suggested by Steven Bolling, proposes simple mitral annuloplasty whatever the underlying cause (primary or ischaemic cardiomyopathy) with symptomatic improvement and better haemodynamics in terms of increased cardiac output and oxygen consumption on exercise and an actuarial survival much higher than that of cardiac transplantation at one and at two years. The most recent innovation is the Batista procedure which is a method of ventricular reduction associated with correction of mitral

  4. Clinical profile of parkinsonism and Parkinson's disease in Lagos, Southwestern Nigeria

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    Ojo Oluwadamilola O


    Full Text Available Abstract Background Current data on the pattern of parkinsonism and Parkinson's disease in Nigerians are sparse. This database was designed to document the clinical profile of PD in Nigerians, and compare this to prior observations. Methods A database of patients presenting to the Neurology out-patients clinic of the Lagos University Teaching Hospital was established in October 1996. Demographic and clinical data at presentation (disease stage using Hoehn and Yahr scale; 'off' state severity on the Unified Parkinson's disease Rating Scale were documented for patients diagnosed with parkinsonism between October 1996 and December 2006. Cases were classified as Parkinson's disease or secondary parkinsonism (in the presence of criteria suggestive of a secondary aetiology. Results The hospital frequency of parkinsonism (over a 2-year period, and relative to other neurologic disorders was 1.47% (i.e. 20/1360. Of the 124 patients with parkinsonism, 98 (79.0% had PD, while 26 (21.0% had secondary parkinsonism. Mean age (SD at onset of PD (61.5 (10.0 years was slightly higher than for secondary parkinsonism (57.5 (14.0 years (P = 0.10. There was a male preponderance in PD (3.3 to 1 and secondary parkinsonism (2.7 to 1, while a positive family history of parkinsonism was present in only 1.02% (1/98 of PD. There was a modestly significant difference in age at onset (SD of PD in men (60.3 (10.4 compared to women (65.2 (7.9 (T = 2.08; P = 0.04. The frequency of young onset PD (≤ 50 years was 16.3% (16/98. The mean time interval from onset of motor symptoms to diagnosis of PD was 24.6 ± 26.1 months with majority presenting at a median 12 months from onset. On the H&Y scale, severity of PD at presentation was a median 2.0 (range 1 to 4. PD disease subtype was tremor-dominant in 31 (31.6%, mixed 54 (55.1% and akinetic-rigid 14 (14.3%. Hypertension was present as a co-morbidity in 20 (20.4%, and diabetes in 6 (6.12%. Conclusions The clinical profile of PD in

  5. Mineralized Remains of Morphotypes of Filamentous Cyanobacteria in Carbonaceous Meteorites (United States)

    Hoover, Richard B.


    The quest for conclusive evidence of microfossils in meteorites has been elusive. One difficulty arises from the fact than many abiotic or inorganic microstructures, mineral grains, and coating artifacts can mimic the smaller representatives of the microbial world that possess very simple morphologies (unicellular cocci or bacilli). However, there exist a wide variety of large, filamentous trichomic prokaryotic microorganisms (cyanobacteria and sulfur bacteria) with sufficiently well known and complex morphologies that they can be recognized and are known to be of unquestionable biogenic origin. The taphonomic modes of fossilization and their of their life habits and processes frequently result in distinctive chemical biosignatures associated with carbonization, silicification, calcification, phosphatization and metal-binding properties of their cell-walls, trichomes, sheaths and extracellular polymeric substances (EPS). Strong differences of mineral concentrations in closely associated and visibly differentiated cellular microstructures provide strong evidence of biogenicity. This evidence is further enhanced by the detection of recognizable and distinct microstructures (e.g., uniseriate or multiseriate filaments, trichomes, sheaths, cells of proper sizes and size distributions) and growth characteristics (e.g., basal or apical cells, true or false branching of trichomes, tapered or uniform filaments, robust or thin sheaths) and reproductive and nitrogen fixation habits (e.g., baeocytes, hormogonia, akinetes and heterocysts), Microfossils of cyanobacteria and cyanobacterial mats and stromatolites have been recognized a described from many of the most ancient rocks on Earth. The crucial problem lies in developing valid protocols and methodologies for establishing that the putative microfossils are truly indigenous and not merely recent microbial contaminants. During the past several years, we have conducted Field Emission Scanning Electron Microscopy (FESEM

  6. Fossils of Cyanobacteria in CI1 Carbonaceous Meteorites: Implications to Life on Comets, Europa and Enceladus (United States)

    Hoover, Richard B.


    Environmental (ESEM) and Field Emission Scanning Electron Microscopy (FESEM) investigations of the internal surfaces of the CI1 Carbonaceous Meteorites have yielded images of large complex filaments. The filaments have been observed to be embedded in freshly fractured internal surfaces of the stones. They exhibit recognizable features (e.g., the size and size ranges of the internal cells and their location and arrangement within sheaths) that are diagnostic of known genera and species of filamentous trichomic cyanobacteria and other trichomic prokaryotes (such as filamentous sulfur bacteria). ESEM and FESEM studies of living and fossil cyanobacteria show features similar to the filaments found in the meteorites -- uniseriate and multiseriate, branched or unbranched, isodiametric or tapered, polarized or unpolarized filaments with trichomes encased within thin or thick external sheaths. Some of the filaments found in the CI1 meteorites also exhibit specialized cells and structures used by cyanobacteria for reproduction (baeocytes, akinetes and hormogonia), nitrogen fixation (basal, intercalary or apical heterocysts), attachment (pili or fimbriae) or indicative of oscillatoria type locomotion (escaped or coiling hormogonia and flattened and coiled empty sheaths). Energy dispersive X-ray Spectroscopy (EDS) studies indicate that the Orgueil meteorite filaments are typically carbon-rich sheaths infilled with magnesium sulfate and other minerals characteristic of the CI1 carbonaceous meteorites. However, the size, structure, detailed morphological characteristics and chemical compositions of the meteorite filaments are not consistent with known species of abiotic minerals. The nitrogen content of the meteorite filaments are almost always below the detection limit of the EDS detector. EDS analysis of living and dead biological materials (e.g., filamentous cyanobacteria; bacteria, mummy and mammoth hair and tissues, and fossils of cyanobacteria, trilobites and insects in

  7. Ballistic Protons and Microwave-induced Water Solitons in Bioenergetic Transformations

    Directory of Open Access Journals (Sweden)

    Reuven Tirosh


    Full Text Available Active streaming (AS of liquid water is considered to generate and overcomepressure gradients, so as to drive cell motility and muscle contraction by hydrauliccompression. This idea had led to reconstitution of cytoplasm streaming and musclecontraction by utilizing the actin-myosin ATPase system in conditions that exclude acontinuous protein network. These reconstitution experiments had disproved a contractileprotein mechanism and inspired a theoretical investigation of the AS hypothesis, aspresented in this article. Here, a molecular quantitative model is constructed for a chemicalreaction that might generate the elementary component of such AS within the pure waterphase. Being guided by the laws of energy and momentum conservation and by the physicalchemistry of water, a vectorial electro-mechano-chemical conversion is considered, asfollows: A ballistic H+ may be released from H2O-H+ at a velocity of 10km/sec, carrying akinetic energy of 0.5 proton*volt. By coherent exchange of microwave photons during 10-10sec, the ballistic proton can induce cooperative precession of about 13300 electrically-polarized water molecule dimers, extending along 0.5 μm. The dynamic dimers rearrangealong the proton path into a pile of non-radiating rings that compose a persistent rowing-likewater soliton. During a life-time of 20 msec, this soliton can generate and overcome amaximal pressure head of 1 kgwt/cm2 at a streaming velocity of 25 μm/sec and intrinsicpower density of 5 Watt/cm3. In this view, the actin-myosin ATPase is proposed to catalyzestereo-specific cleavage of H2O-H+ , so as to generate unidirectional fluxes of ballisticprotons and water solitons along each actin filament. Critical requirements and evidentialpredictions precipitate consistent implications to the physical chemistry of water, enzymatichydrolysis and synthesis of ATP, trans-membrane signaling, intracellular transport, cellmotility, intercellular interaction, and associated

  8. Neuroacanthocytosis. A clinical, haematological and pathological study of 19 cases. (United States)

    Hardie, R J; Pullon, H W; Harding, A E; Owen, J S; Pires, M; Daniels, G L; Imai, Y; Misra, V P; King, R H; Jacobs, J M


    Nineteen cases are described, including 12 cases from three different families and 7 nonfamilial cases, in which multisystem neurological disease was associated with acanthocytosis in peripheral blood and normal plasma lipoproteins. Mild acanthocytosis can easily be overlooked, and scanning electron microscopy may be helpful. Some neurologically asymptomatic relatives with significant acanthocytosis were identified during family screening, including some who were clinically affected. The mean age of onset was 32 (range 8-62) yrs and the clinical course was usually progressive but there was marked phenotypic variation. Cognitive impairment, psychiatric features and organic personality change occurred in over half the cases, and more than one-third had seizures. Orofaciolingual involuntary movements and pseudobulbar disturbance commonly caused dysphagia and dysarthria that was sometimes severe, but biting of the lips or tongue was rarely seen. Chorea was seen in almost all symptomatic cases but dystonia, tics, involuntary vocalizations and akinetic-rigid features also occurred. Two cases had no movement disorder at all. Computerized tomography often demonstrated cerebral atrophy. Caudate atrophy was seen less commonly, and nonspecific focal and symmetric signal abnormalities from the caudate or lentiform nuclei were seen by magnetic resonance imaging in 3 out of 4 cases. Depression or absence of tendon reflexes was noted in 13 cases and neurophysiological abnormalities often indicated an axonal neuropathy. Sural nerve biopsies from 3 cases showed evidence of a chronic axonal neuropathy with prominent regenerative activity, predominantly affecting the large diameter myelinated fibres. Serum creatine kinase activity was increased in 11 cases but without clinical evidence of a myopathy. Postmortem neuropathological examination in 1 case revealed extensive neuronal loss and gliosis affecting the corpus striatum, pallidum, and the substantia nigra, especially the pars

  9. Left ventricle remodelling by double-patch sandwich technique

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    Nocchi Andrea


    Full Text Available Abstract Background The sandwich double-patch technique was adopted as an alternative method for reconstruction of the left ventricle after excision of postinfarction dysfunctional myocardium to solve technical problems due to the thick edges of the ventricular wall. Methods Over a 5-year period, 12 of 21 patients with postinfarction antero-apical left ventricular aneurysm had thick wall edges after wall excision. It was due to akinetic muscular thick tissue in 6 cases, while in the other 6 with classic fibrous aneurysm, thick edges remained after the cut of the border zone. The ventricular opening was sandwiched between two patches and this is a technique which is currently used for the treatment of the interventricular septum rupture. In our patients the patches are much smaller than the removed aneurysm and they were sutured simply by a single row of single stitches. However, in contrast to interventricular septum rupture where the patches loosen the tension of the tissues, in our patients the patches pull strongly and restrain the walls by fastening their edges and supporting tight stitches. In this way they could narrow the cavity and close the ventricle. Results The resected area varied from 5 × 4 to 8 × 8 cm. Excision was extended into the interventricular septum in 5 patients, thus opening the right ventricle. CABG was performed on all patients but two. Left ventricular volumes and the ejection fraction changed significantly: end-systolic volume 93.5 ± 12.4 to 57.8 ± 8.9 ml, p Conclusion The double-patch sandwich technique (bi-patch closure offers some advantages and does not result in increased morbidity and mortality. In the case of excising a left ventricular aneurysm, this technique in no way requires eversion of the edges, felt strips, buttressed and multiple sutures, all of which are needed for longitudinal linear closure. Moreover, it does not require purse string sutures, endocardial scar remnant to secure the patch or

  10. Principal stages in evolution of precambrian organic world: Communication 2. The late proterozoic (United States)

    Sergeev, V. N.; Semikhatov, M. A.; Fedonkin, M. A.; Vorob'eva, N. G.


    A new suggested model outlining the evolution of the organic world from the mid-Early Proterozoic (˜2.0 Ga) to the Early Cambrian is based on data characterizing the relevant chert-embedded and compression-preserved organic-walled microbiotas, impressions of soft-bodied multicellular organisms, and biomarkers. Critical analysis of overall paleontological data resulted in the distinguishing of seven successive assemblages of Proterozoic micro- and macrofossils. Being of global geographic range, the assemblages correspond to the major stages in evolution of the organic world and typify global units which are termed the Labradorian (˜2.0-1.65 Ga), Anabarian (1.65-1.2 Ga), Turukhanian (1.2-1.03 Ga), Uchuromayan (1.03-0.85 Ga), Yuzhnouralian (0.85-0.635 Ga), Amadeusian (0.635-0.56 Ga), and Belomorian (0.56-0.535 Ga). Characteristic of the Labradorian unit are microfossil assemblages of the Gunflint type including remains of morphologically bizarre prokaryotic microorganisms: star-like Eoastrion, umbrella-shaped Kakabekia, dumbbell-shaped Xenothrix, and some others. Fine-grained siliciclastic deposits of the same age yield the oldest remains of millimeter-sized eukaryotes: spherical to ribbon-like Chuaria and Tawuia. Microfossils prevailing in shallow-water carbonate facies of the Anabarian unit are akinetes of nostocalean cyanophyceae Archaeoellipsoides and entophysalidacean cyanobacteria Eoentophysalis, whereas acanthomorphic acritarchs Tappania and Shuiyousphaeridium dominate the assemblages of open-shelf facies, where they are associated with the first-found rare macroscopic multicellular fossils Horodyskia. The distinguishing feature of the next Turukhanian unit is the first occurrence of filamentous red alga Bangiomorpha and the stalked cyanobacterium Polybessurus. The Uchuromayan unit is characterized by the appearance and worldwide radiation of structurally complicated eukaryotic microorganisms, primarily of acanthomorphic acritarchs Trachyhystrichosphaera and

  11. [Regional ventricular function at rest during exercise before and after bypass surgery (author's transl)]. (United States)

    Bussmann, W D; Mayer, V; Kober, G; Kaltenbach, M


    In 9 patients with coronary heart disease isovolumetric contractility indices and ejection phase parameters were measured simultaneously, using an angiographic catheter with a manometer at the tip (Millar). Regional wall motion at rest, after leg raising and during physical exercise (bicycle ergometer) was analyzed applying the hemiaxis method. Five weeks after aortocoronary bypass surgery the same examinations were repeated. Preoperatively left ventricular enddiastolic pressure (LVEDP) increased from 21 to 37 mm Hg following leg raising. The velocity mean of fiber shortening (Vcf) and of regional fiber shortening in the anterior wall decreased significantly.--All patients discontinued physical exercise due to angina pectoris. LVEDP increased from 21 to 39 mm Hg. Large hypokinetic and akinetic areas developed especially in the anterior wall. Velocity of fiber shortening of the anterior wall decreased from 1.43 to 0.76/s. Enddiastolic volume remained unchanged while endsystolic volume increased significantly. In six patients with patent grafts surgery had a beneficial effect. Comparing angiograms at rest no significant changes were found. After leg raising and physical exercise, however, marked improvement in ventricular function occurred, compared to the preoperative performance. All 6 patients were exercised without complaints at a load of 100 watts for 8 minutes. Velocity of fiber shortening in the anterior wall increased significantly from 0.76 to 2.56/s, mean Vcf from 1.11 to 2.12 circ/s, max dP/dt from 2302 to 4280 mm Hg/s and Vpm from 27.8 to 55.7/s. Functional improvement in individual wall segments amounted to 500% in the mean. Ejection fraction increased from 54 to 76%. Enddiastolic volume remained unchanged while endsystolic volume decreased from 67 to 33 ml/1.37 m2 (p less than 0.002). In three patients the bypass occluded or myocardial infarction occurred intraoperatively. Postoperative findings at rest and during exercise were unchanged as compared to

  12. Percutaneous Ventricular Restoration Therapy Using the Parachute Device in Chinese Patients with Ischemic Heart Failure: Three-Month Primary End-point Results of PARACHUTE China Study

    Institute of Scientific and Technical Information of China (English)

    Yue-Jin Yang; Yong Huo; Ya-Wei Xu; Jian-An Wang; Ya-Ling Han; Jun-Bo Ge; Rui-Yan Zhang


    Background:The primary cause of ischemic heart failure (HF) is myocardial infarction (MI) resulting in left ventricle (LV) wall motion abnormality secondary to ventricular remodeling.A prospective,nonrandomized study conducted in China was designed to assess safety and efficacy of the percutaneous ventricular restoration therapy using Parachute device (CardioKinetix,Inc.,CA,USA) in ischemic HF patients as a result of LV remodeling after anterior wall MI.Methods:Thirty-one patients with New York Heart Association (NYHA) Class Ⅱ,Ⅲ ischemic HF,ejection fraction between 15% and 40%,and dilated akinetic or dyskinetic anterior-apical wall without the need to be revascularized were enrolled from seven sites in China from October to December 2014.The Parachute device was implanted through femoral artery.All patients received low-dose aspirin and anticoagulation with warfarin for at least 12 months postdevice implantation.The primary end-point was the assessment of efficacy as measured by the reduction in LV end-systolic volume index (LVESVI) against baseline LVESVI at 3 months postdevice implantation,determined by the echocardiography and measured by echocardiography core laboratory.Quality of life was assessed using EQ-5D and visual analog scale (VAS).For quantitative data comparison,paired t-test (normality data) and signed-rank test (abnormality data) were used;application of signed-rank test was for the ranked data comparison.Results:A change in LVESVI as measured by echocardiography from the preimplant baseline to 3-month postdevice implantation revealed a statistically significant reduction from 77.5 ± 20.0 ml/m2 to 53.1 ± 17.0 ml/m2 (P < 0.0001).The trial met its primary end-point.Of the 31 patients,the procedural success was 96.8%.Overall,NYHA HF class assessment results showed an improvement of more than halfa class at 3 months (P < 0.001).Quality of life assessed by the VAS value increased 11.5 points (P < 0.01),demonstrating improvement at 3

  13. A putative O-linked β-N-acetylglucosamine transferase is essential for hormogonium development and motility in the filamentous cyanobacterium Nostoc punctiforme. (United States)

    Khayatan, Behzad; Bains, Divleen K; Cheng, Monica H; Cho, Ye Won; Huynh, Jessica; Kim, Rachelle; Omoruyi, Osagie H; Pantoja, Adriana P; Park, Jun Sang; Peng, Julia K; Splitt, Samantha D; Tian, Mason Y; Risser, Douglas D


    including nitrogen-fixing heterocysts, spore-like akinetes, and motile hormogonia that function in dispersal as well as the establishment of nitrogen-fixing symbioses with plants and fungi. These symbioses are major contributors to global nitrogen fixation. Despite the fundamental importance of hormogonia to the life cycle of filamentous cyanobacteria and the establishment of symbioses, the molecular regulation of hormogonium development is largely undefined. We employed a genetic screen to identify genes essential for hormogonium development and motility in Nostoc punctiforme The first gene identified using this screen encodes a eukaryotic-like O-linked β-N-acetylglucosamine transferase that is required for accumulation of PilA in hormogonia.

  14. Enfermedad de Creutzfeldt-Jakob por RMI: alteración cortical como signo temprano de la enfermedad Creutzfeldt-Jakob disease by MRI: Cortical alteration as early sign disease

    Directory of Open Access Journals (Sweden)

    María Fernanda Markarian


    Full Text Available Se estudió por RMI un paciente de 59 años con diagnóstico probable de Enfermedad de Creutzfeldt-Jakob desde el inicio de sus síntomas. El paciente comienza con un cuadro de leve deterioro cognitivo. En una primera resonancia en secuencias FLAIR se visualiza hiperintensidad cortical a predomino de hemisferio izquierdo, no observándose en FSE T 2. Se hace más significativa en nueva resonancia en FLAIR y Difusión, con aparición de hiperintensidad en cabeza de ambos caudados y rápido deterioro cognitivo, alteraciones visuales, aparición de signos piramidales y extrapiramidales, convulsiones y mioclonias y mutismo. Con la acentuación de las alteraciones corticales -y en los ganglios de la base en una tercera resonancia-, el paciente trasforma su ECG de ritmo lento a un ritmo de punta-onda bifásico y trifásico. A 3 meses de la primera resonancia, nuevas imágenes muestran atrofia e importante hiperintensidad cortical y en ganglios de la base. En conclusión, las secuencias FLAIR y Difusión serían más sensibles que las secuencias T2 en la detección del aumento de intensidad de señal en la corteza cerebral, siendo un indicio diagnóstico temprano de la enfermedad de Creutzfeld-Jakob.A 59-year-old man with probable Creutzfeldt-Jakob disease was studied from early symptoms. The patients manifested mild cognitive impairment. The first magnetic resonance showed hiperintense signal cortical abnormalities in FLAIR sequence predominantly in left hemisphere, FSE T2 no showed abnormalities. In other resonance those abnormities were more significative and appeared head of the caudate nucleus abnormalities in FLAIR and Diffusion-weighted, the patients began with rapidly progressing impairment, visual disturbance, pyramidal and extrapyramidal signs, seizures, myoclonus and mutism. The third resonance revealed cortical and basal ganglia high signal intensity abnormalities and the patient transformed slowing EEG to biphasic and triphasic sharp

  15. Neurological Effects of Acute Carbon Monoxide Poisoning in Children

    Directory of Open Access Journals (Sweden)

    Coskun YARAR


    Full Text Available Carbon monoxide poisoning (COP is one of the most common causes of mortality and morbidity due to poisoning in all over the world. Although the incidence of COP has not been known exactly in the childhood, almost one-third of CO exposures occurred in children. The data regarding COP in children are inconclusive. Children may be more vulnerable to CO exposure than adults as a result of their high respiration and metabolic rates, high oxygen metabolism, and immature central nervous system. Recent researches proposed new theories about neurological effects of CO toxicity. The clinical presentations associated acute COP may be various and nonspecific. Unrecognized CO exposure may lead to significant morbidity and mortality. CO exposed children often become symptomatic earlier, and recover more rapidly, than similarly CO exposed adults. Mild clinical signs and symptoms associated with COP are headache, dizziness, weakness, lethargy, and myalgia; however, severe signs and symptoms such as blurred vision, syncope, convulsion, coma, cardiopulmonary arrest and death can also accompany with COP. Neurologic manifestations can include altered mental status at different degrees, neck stiffness, tremor, ataxia, and positive Babinski's sign. Delayed neurologic sequels (DNS of COP might be seen in children like adults. DNS symptoms and signs in children include memory problems, mental retardation, mutism, fecal and urinary incontinence, motor deficits, facial palsy, psychosis, chronic headache, seizures, and epilepsy. After CO exposure children must be cared to detect and treat DNS. Although hyperbaric oxygen therapy (HBOT is reported to prevent development of DNS, its indications, application duration and procedures are controversial in both of the children and adults. Although their predictive values are limited, exposing to CO more than eight hours and suffering from CO-induced coma, cardiac arrest, lactic acidosis, high COHb levels, and pathologic findings


    Institute of Scientific and Technical Information of China (English)

    王丹蕾; 聂桂平


    This article presents the social background of sign language translator design at the beginning. Then it introduces two kinds of gesture recognition technologies used by most sign language translator today, and analyzes some typical products for each of the technologies to comprehend their advantages and disadvantages, and enumerates other new gesture recognition technology which can be used for sign language translator design as wel. Secondly, the article analyzes the design of sign language translator from aspects of user requirements, product form, emotion experience, and points out the shortages of the existing design .It also put forward some advices for the design of sign language translator in future. Article believes that the sign language translator should not only offset the deficiency caused by the absence of organs, but also should pay attention to the emotional needs of the deafness-mutism.%文章以手语翻译器设计的社会背景开篇,首先介绍了现有的手语翻译器所使用的两类手势识别技术,选取了各类中具有代表性的设计进行了详细介绍并分析其利弊,又对其他可应用的手势识别技术做了补充。然后,从用户需求、产品形式、情感体验三个方面对现有的手语翻译器设计进行了分析,指出了现有设计的不足并提出了改进的方向。文章认为,未来的手语翻译器应该在满足器质性需求的同时关注听力言语障碍人士的情感需求,既能帮助他们传递信息又能帮助他们传递情感。

  17. Surgical considerations for ′intrinsic′ brainstem gliomas: Proposal of a modification in classification

    Directory of Open Access Journals (Sweden)

    Mehta V


    Full Text Available Background: Brainstem gliomas are highly heterogeneous tumors both in their clinical manifestation and in their pathology. Despite significant advances in the surgery for brainstem gliomas many aspects of this pathology are still unclear Objective: To evaluate the clinical, radiological and surgical outcome of 40 focal ′intrinsic′ brainstem gliomas and propose a surgical strategy-oriented classification. Materials and Methods: A total of 40 focal ′intrinsic′ ("expanding variety" tumors have been operated over a period of 8.5-years (January 1998-June 2007. Our criteria included patients with (1 well-defined gadolinium enhancing tumor; (2 relatively long duration of symptoms (> six months and (3 good neurological functional status and independent for all activities of daily living. The cutoff size of 2 cm was not rigidly adhered to. Results: The ′intrinsic′ brainstem tumors were classified into three types: Expanding, diffuse infiltrative and pure ventral varieties. Only patients with expanding variety of brainstem gliomas were subjected to surgery, mean age 19.2 years (range 4-55 years and male to female ration mean: 3:2. The tumor location included pons (n=19, midbrain (n=13 and medulla (n=8. Surgical approaches included midline suboccipital (n=28, retromastoid (n=7, subtemporal (n=3 and supracerebellar-infratentorial (n=2. Thirty-two cases with ′diffuse infiltrative′ and ′pure ventral′ variety were given radiotherapy only. Histology pathology revealed pilocytic variety (n=10, Grade II (n=17 and Grade III (n=13. There was one death in the surgical series (due to aspiration. Complications included meningitis (n=2, wound infection (n=1, chest infection (n=5 and transient mutism (n=1. Follow-up ranged from 3-68 months. Overall, 36 improved /remained same and three worsened in their clinical status at the time of discharge. Conclusion: The surgical management of intrinsic brainstem tumors presents a surgical challenge; radical

  18. [Impact of DSM-5: Application and Problems Based on Clinical and Research Viewpoints on Anxiety Disorders]. (United States)

    Shioiri, Toshiki


    In Japan, the impact of DSM-5 has been greater than we had imagined. The Japanese Society of Psychiatry and Neurology organized a group for translation and the members spent many hours in this volunteer effort over a 2-year period. This highlights the significance of and expectations for DSM-5 in clinical practice in Japan. Regarding anxiety disorders, the highlights of changes from DSM-IV-TR to DSM-5 are as follows. Firstly, the DSM-5 chapter on anxiety disorder no longer includes obsessive-compulsive disorder (which is included with obsessive-compulsive and related disorders) or posttraumatic stress disorder and acute stress disorder(which are included with trauma- and stressor-related disorders). However, the sequential order of these chapters in DSM-5 reflects the close relationships among them. Secondly, in DSM-IV, selective mutism and separation anxiety disorder were classified in the section "Disorders Usually First Diagnosed in Infancy, Childhood, or Adolescence." They are now classified as an anxiety disorder. Through these two changes, at the beginning of the chapter, it can be clearly noted that anxiety disorders include disorders that share features of excessive fear and anxiety and related behavioral disturbances. Thirdly, panic disorder and agoraphobia are not associated in DSM-5. Thus, the former DSM-IV diagnoses of panic disorder with agoraphobia, panic disorder without agoraphobia, and agoraphobia without a history of panic disorder are now replaced by two diagnoses, panic disorder and agoraphobia, each with separate criteria. The co-occurrence of panic disorder and agoraphobia is now coded with two diagnoses. This change recognizes that a marked number of individuals with agoraphobia do not experience panic symptoms. For the present, this change ends the. controversy over the hierarchy between panic disorder and agoraphobia. The diagnostic criteria for agoraphobia are derived from the DSM-IV descriptors for agoraphobia, although the clarification

  19. 经胼胝体-穹窿间切除第三脑室松果体区肿瘤%Transcallosal-interfornix approach to remove the tumors of the third ventricle and pineal region

    Institute of Scientific and Technical Information of China (English)

    叶劲; 钟书; 梁有明; 肖泉; 刘若平; 张玉琪; 马振宇


    目的 探讨经胼胝体-透明隔-穹窿间入路显微手术切除第三脑室松果体区肿瘤的方法及疗效,并对相应的显微解剖学基础加以讨论.方法 经胼胝体-透明隔-穹窿间入路显微手术切除第三脑室松果体区肿瘤16例.结果 术前1例先行脑室-腹腔分流术、1例行神经内镜第三脑室底终板造瘘术.16例在手术显微镜下肿瘤全切除9例,次全切除4例,大部切除3例.5例在处理完肿瘤后做终板造瘘术.结论 经胼胝体-透明隔-穹窿间入路手术切除第三脑室松果体区肿瘤系通过胚胎组织残留的透明隔间隙进入第三脑室,损伤少,肿瘤全切除、次全切除率高,术后并发症少.%Objective To evaluate the efficacy of microsurgical resection of the pineal region tumors of the third ventricle through trans-corpus-callosum approach.Methods From January 2006 to June 2008, sixteen pineal region tumors of the third ventricle were resected through trans-corpus-callosum approach.In sixteen cases, two hypothalamic astroeytoma eases received ventricle shunt before operations;four germ cell tumors and one hypothalamic astrocytoma cases received end-plate-ostomy after operations.Of Sixteen eases, there were 4 astrocytomas,2 ependymomas, 2 central nervous system tumors,1 teratoma,1 cerebral cysticercosis, 6 pineal region tumors(including 4 germ cell tumor, 1 teratoma and 1 dermoid cyst). Results Of 16 microsurgical resections, 9 achieved total resection, 4 subtotal resection, 3 major resection (tumors in third ventricle: 7 achieved total resection, 2 subtotal resection,1 major resection;tumors in pineal region: 2 achieved total resection, 2 subtotal resection, 2 major resection). There was no postoperative coma or death.A postoperative hematoma in ventricle was found in one case with central nervous system tumor and the hematoma was removed.After operation,3 patients could not watch upwards,4 had the last obstacle to memory,1 had mutism. All patients were

  20. 胼胝体全段一期切开治疗癫(癎)性脑病%Single-stage total callosotomy for treatment of epileptic encephalopathy

    Institute of Scientific and Technical Information of China (English)

    李云林; 夏竑; 周震; 王磊; 王晓飞; 郑瑞峰; 刘庆祝; 马康平; 代金东; 张景; 刘卫芳; 陈卉


    operative efficiency was reached to 75. 7% totally and 86. 2% ,77. 5%, 61.6% and 40.8% in different type of seizure,including drop attacks,tonic seizure,spasm and generalized tonic-clonic seizure respectively. Postoperative complications were happened in 14 cases with 11 cases of mutism ,7 cases of paralysis and 1 case of infection. Most of the patients were recovered from the transient complications and only 1 case suffering from permanent paresis. Conclusion Single-stage total CC was a safe, micro-invaded method to reduce the seizure frequency and severity of the patients with epileptic encephalopathy without added complications compared with partial CC.

  1. Are lichens and cyanobacteria suitable candidates to test the theory of lithopanspermia? (United States)

    de La Torre Noetzel, Rosa; Martinez Frías, Jesús; Mateo-Martí, Eva; Sanchez Iñigo, Francisco Javier; García Sancho, Leopoldo; Horneck, Gerda


    Lichens, endolithic- and endoevaporitic communities of cyanobacteria and bacteria has been exposed to real- and simulated space conditions to demonstrate likelihood of the lithopanspermia hypothesis, that postulates a viable transport of microorganisms between planets by means of meteorites, i.e. impact expelled rocks from a planets surface, which serve as vehicles for spreading living material from one planet or solar system to another (Nicholson et al. 2000, Benardini et al. 2003, Cockell 2008, Horneck et al. 2008, Valtonen et al. 2009). Three experiments (LICHENS, Foton M2 mission, 2005, and LITHOPANSPERMIA, Foton-M3 satellite, 2007), have been performed in space, the first two on a short mission, onboard of Biopan of the Foton-M satellite recoverable capsule, and the third one in a long-term mission on the Expose facility of the ISS to test the survival of prokaryotic- and eukaryotic symbiotic organisms in relation to lithopanspermia. The first two experiments allowed for the first time the demonstration- and intercomparison of the high survival capacity of eukaryotic- and prokaryotic symbiotic organisms in space (the epilithic lichen species Rhizocarpon geographicum and Xanthoria elegans, endoevaporitic microbial communities, epilithic microbial communities with cyanobacterial akinetes of Anabaena, and a vagrant lichen species, Aspicilia fruticulosa). Exposure to different UV-conditions of a low Earth orbit (LEO, 300 km) were performed: solar extraterrestrial UV radiation, Mars simulated UV-climate, UV-B radiation and PAR, space vacuum at 10-6 mbar, microgravity and temperatures between -23°C and +16°C. To check the resistance of the selected organisms to space before these missions, space simulation experiments were performed at INTA (Spasolab) and DLR (Institute of Aerospace Medicine), which were decisive to show the high survival capacity of these species to space vacuum (10-4 - 10-6 mbar), space UV radiation (200-400 nm) and extreme temperatures. The

  2. Tratamento cirúrgico dos aneurismas de ventrículo esquerdo e isquemia coronária

    Directory of Open Access Journals (Sweden)

    Danton R. da Rocha LOURES


    reconstruction and their effects on morphology, ventricular function, symptomatic state and longevity. This study was designed to evaluate the cases of left ventricular aneurysm relating the main surgical indications and treatment and the patients' symptomatic state before and after operation. The study involves 12 patients with left ventricular aneurysm, nine males, mean age 60 years, whose main indications for surgery were angina (58% and angina plus congestive heart failure (42%. Ventricular cineangiograms revealed an akinetic/dyskinetic segment in 92% of patients, apical or anteroapical aneurysm in 83% and coronary artery disease in 100%, affecting 3 or more vessels in 75%, the left anterior descending artery was most affected (29%. The ejection fraction ranged from 32% to 66%. All patients underwent coronary artery bypass grafting, nine had left ventricular aneurysmectomy, seven through linear closure and 2 through geometric reconstruction. The cardiopulmonary bypass and aortic cross-clamp mean time was 96 and 50 minutes for linear closure and 180 and 86 for geometric reconstruction. The main complications after surgery were low cardiac output and atrial fibrillation (16%. Hospital mortality rate was 16%. Patients have a mean follow up of 15 months, survival rate is 75%, those surgically treated for CHF are in class II and 89% are angina-free. These improvements in quality of life provide justification for aneurysmectomy.

  3. Dobutamine cine magnetic resonance imaging after myocardial infarction; Cine Risonanza Magnetica con dobutamina dopo infarto del miocardio

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    Giovagnoni, A.; Ligabue, G.; Romagnoli, R. [Modena Univ., Modena (Italy). Scienze Radiologiche, Dipt. di Medicina Interna; Reggio Emilia Univ., Reggio Emilia (Italy). Cattedra di Cardiologia; Rossi, R.; Muia, N.; Modena, M.G. [Modena Univ., Modena (Italy). Dipt. di Scienze Mediche, Oncologiche e Radiologiche; Reggio Emilia Univ. (Italy). Dipt. di Scienze Mediche, Oncologiche e Radiologiche


    Dobutamine Cine MRI is a new diagnostic imaging technique in the pretreatment (revascularization) assessment of myocardial infarction patients. In this issue are reported the result of a comparative study of the diagnostic yield of dobutamine Cine MRI with that of stress echocardiography in the assessment of viable myocardium. A new method for analysis of Cine MR images, employing digital subtraction, aimed at decreasing subjectivity in the quantitative assessment of myocardial wall thickening. Twenty-six patients (21 men and 5 women) with a history of myocardial infarction who were scheduled for revascularization were submitted to stress echocardiography and dobutamine Cine MRI to evaluate contractile recovery of the segments considered akinetic or hypo kinetic at baseline echocardiography. Dobutamine was administered in growing doses (5, 10, 15{gamma}/kg/min). 16 segments of the left ventricle in each patient were considered. In the 416 segments studied, it was found that 307 normo kinetic, 64 scarred and 45 viable segments with stress echocardiography, versus 302 normo kinetic, 83 scarred and 31 viable segments with dobutamine MRI. Three months after revascularization 15 patients were examined to check contractile recovery of the segments considered as viable. Echocardiography had 79% sensitivity and 97% specificity, while Cine MRI had 96% and 86%, respectively. In patients with anteroseptal wall myocardial infarction stress echocardiography had 75% sensitivity and 97% specificity. Echocardiography permits to distinguish viable myocardium and scarred myocardial tissue with good sensitivity and specificity, but Cine MRI performs better. Cine MRI has much higher sensitivity than stress echocardiography and thus makes the technique of choice to evaluate viable myocardium in these sites. The digital subtraction technique is as accurate as manual measurements, but reduces the error rate and permits quicker evaluation, particularly in subendocardial thickening

  4. [Catatonia: resurgence of a concept. A review of the international literature]. (United States)

    Pommepuy, N; Januel, D


    Catatonia was first described in 1874 by Kahlbaum as being a cyclic disease mixing motor features and mood variations. Because most cases ended in dementia, Kraepelin recognized catatonia as a form of dementia praecox and Bleuler included it within his wide group of schizophrenias. This view influenced the psychiatric practice for more than 70 years. But catatonia was recently reconsidered and this because of the definition of more precise diagnosis criteria, the discovery of a striking association with mood disorders, and the emphasis on effective therapeutics. Peralta et al empirically developed a performant diagnostic instrument with the 11 most discriminant signs among catatonic features. Diagnostic threshold is three or more signs with sensitivity of 100% and specificity of 99%. These signs are: immobility/stupor (extreme passivity, marked hypokinesia); mutism (includes inaudible whisper); negativism (resistance to instructions, contrary comportment to whose asked); oppositionism, other called gegenhalten (resistance to passive movement which increases with the force exerted); posturing (patient adopts spontaneously odd postures); catalepsy (patient retains limb positions passively imposed during examination; waxy flexibility); automatic obedience (exaggerated co-operation to instructed movements); echo phenomena (movements, mimic and speech of the examiner are copied with modification and amplifications); rigidity (increased muscular tone); verbigeration (continuous and directionless repetition of single words or phrases); withdrawal/refusal to eat or drink (turning away from examiner, no eye contact, refusal to take food or drink when offered). Using this diagnostic tool, prevalence of catatonic syndrome appears to be close to 8% of psychiatric admissions. Other signs are also common but less specific: staring, ambitendance, iterations, stereotypes, mannerism, overactivity/excitement, impulsivity, combativeness. Some authors complete this description by

  5. 脆骨病一家系4代的调查分析%Investigation and Analysis on the 4 Generation of Brittle Bone Disease Family

    Institute of Scientific and Technical Information of China (English)

    谷国俊; 王新家


    Objective: To study the inheritance patterns and clinical features of a clinical osteogenesis imperfecta (OI) family.Method: To make interview and phone investigation of bone family members (clinical questionnaire about medical history, physical examination etc.), and collect the clinical data of the 4-generation family with 45 members in all (including gender, age, height, medical history and the main characteristics and signs), then draw the family genetic map, summarize and analyze the clinical features and genetic methods of the OI family.Result: (1) The clinical features:among the 45 members of the investigated family, there were 7 patients suffered from type Ⅰ OI, whose ratio of the male and female was 2:5, including 7 cases of blue sclera, 4 cases of fracture, 4 cases of incomplete dentin formation, and 1 case of liver cancer, who had died. Meanwhile, there were 2 cases of congenital deaf mutism, who were non osteogenesis imperfecta patients. (2) The family map showed that the genetic way of incomplete osteogenesis was consistent with autosomal dominant inheritance.Conclusion: (1) The diagnosis of O pedigree I was consistent with type Ⅰ OI, the mode of inheritance was autosomal dominant inheritance, and sporadic cases caused by gene mutation. So once it was gotten, it will be inherited in a certain way, bringing diseases to offspring and become genetic familial incidence. (2) There were 100% blue sclera in typeⅠ osteogenesis imperfecta patients, and it can be in a separate genetic, without gristle performance. What’s more, the depths of the color can reflect seriousness of the disease. (3) Correct treatment of fracture and protection measures could avoid or reduce fractures. In addition, cautious should be taken on prenatal diagnosis to reduce the birth of OI patients.%目的:探讨临床发现的一成骨不全(OI)家系的遗传方式及临床特点。方法:对发现的成骨不全家系进行电话和现场调查(问卷调查病史、

  6. Microsurgical anatomy and clinical application of infratentorial supracerebellar keyhole approach%小脑幕下小脑上锁孔入路的显微解剖研究及其初步临床应用

    Institute of Scientific and Technical Information of China (English)

    兰青; 朱玉辐; 贡志刚


    and pineal region,based on the individualized imaging features.Results Only the central group bridging veins on the cerebellum tentorial surface needed to be sacrificed under infratentorial supracerebellar keyhole approach,and other vessels could be preserved well The distance between the bridging veins of beth left and right medial group could reach (35±6) mm when they were fully dissected,thus offering enough surgical space for applying this keyhole approach.When the culmen of cerebellum was retracted,the pineal body,Galen vein and its tributary,quadrigeminal bodies,even trochlear nerve could be observed.When the underlayer choroid of the third ventricular roof was dissected medially and inferiorly to the posterior choroidal artery,the posterior third ventricle could be reached.If the tentorium of cerebellum was cut open between the straight sinus and the lateral sinus,the surgical field could be enlarged anteriorly and superiorly to the longitudinal fissure region above the tentorium.Eight of the 11 pineal region tumors sized 2 -5 cm in diameter were totally removed,and 3 were subtotally resected.For one case,posterior cranial fossa decompression and ventriculoperitoneal shunt were performed postoperatively.Another one patient suffered from transient mutism and steady fixation after surgery.Other patients had no adverse effect.Conclusions Infratentorial supracerebellar keyhole approach can be applied for removing the pineal region minors without interfering the adjacent deep venous system,and can enter the third ventricle in a nearly non-traumatic way.If necessary the tentorium can be cut open to expose supra-tentorial structures.It can be regarded as a better choice for surgeries in this region.

  7. Total allowable concentrations of monomeric inorganic aluminum and hydrated aluminum silicates in drinking water. (United States)

    Willhite, Calvin C; Ball, Gwendolyn L; McLellan, Clifton J


    toxicity is also manifest in the hematopoietic system as an erythropoietin-resistant microcytic hypochromic anemia. Signs of Al toxicity in the central nervous system (speech difficulty to total mutism to facial grimacing to multifacial seizures and dyspraxia) are related to Al accumulation in the brain and these symptoms can progress to frank encephalopathy. There are four groups of people at elevated risk of systemic Al intoxication after repeated ingestion of monomeric Al salts: the preterm infant, the infant with congenital uremia and children and adults with kidney disease. There is a dose-dependent increase in serum and urinary Al in people with compromised renal function, and restoration of renal function permits normal handling of systemically absorbed Al and resolution of Al bone disease. Clinical experience with 960 mg/day of Al(OH)(3) (~5 mg Al/kg-day) given by mouth over 3 months to men and women with compromised renal function found subclinical reductions in hemoglobin, hematocrit and serum ferritin. Following adult males and females with reduced kidney function found that ingestion of Al(OH)(3) at 2.85 g/day (~40 mg/kg-day Al) over 7 years increased bone Al, but failed to elicit significant bone toxicity. There was one report of DNA damage in cultured lymphocytes after high AlCl(3) exposure, but there is no evidence that ingestion of common inorganic Al compounds presents an increased carcinogenic risk or increases the risk for adverse reproductive or developmental outcomes. A number of studies of Al exposure in relation to memory in rodents have been published, but the results are inconsistent. At present, there is no evidence to substantiate the hypothesis that the pathogenesis of Alzheimer's Disease is caused by Al found in food and drinking water at the levels consumed by people living in North America and Western Europe. Attapulgite (palygorskite) has been used for decades at oral doses (recommended not to exceed two consecutive days) of 2,100

  8. Stem Cell Therapy for Congestive Heart Failure

    Directory of Open Access Journals (Sweden)

    Gunduz E


    engraft in sufficient numbers to differentiate to the cardiac myocytes and restore functionality in these akinetic areas. Hematopoietic stem cells consist can differentiate to skeletal and myocardial cells when cultured under appropriate conditions [4]. Strauer et al [5] reported that intracoronary bone marrow stem cell therapy improves ventricular performance, quality of life and survival in patients with chronic heart failure. Hamano et al [6] showed in 5 patients that autologous bone marrow cells can be injected safely during a by-pass operation into areas of ischemic myocardium. Brehm et al [7] have treated 23 patients with acute cardiac infarction using autologous mononuclear bone marrow cells. Ozbaran et al [8] transplanted peripheral blood stem cells into areas of injury with open-heart surgery in six patients with ischemic cardiomyopathy. There are also meta-analysis on ongoing clinical trials performed. Abdel-Latif et al [9] described a statistically significant improvement in ejection fraction, reduction in infarct size and left ventricular end-systolic volume in 18 patients treated with either unseparated bone marrow cells, bone marrow mesenchymal and mobilized peripheral blood cells. Martin-Rendon et al ­­ focused on 13 randomized studies encompassing 811 participants on bone marrow therapy for post acute infarction. Improvement in LVEF, decrease in left ventricular and systolic and end diastolic volumes and infarct size were observed. The reason for choice of intravenous route was being the standard way of giving hematopoietic stem cells by hematologists. We tried to decide the availibility of this route for indications other than hematological diseases. Although trapping of stem cells in the pulmonary vascular bed is a drawback of intravenous route and the question of whether the patient would have been more benefited by intracoronary or intramyocardial route remains unanswered, we think intravenous route may still have some role according to our own