Selective mutism and temperament: the silence and behavioral inhibition to the unfamiliar.

Science.gov (United States)

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

2016-10-01

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 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.

Treatment of Selective Mutism: A Best-Evidence Synthesis.

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Stone, Beth Pionek; Kratochwill, Thomas R.; Sladezcek, Ingrid; Serlin, Ronald C.

2002-01-01

Presents systematic analysis of the major treatment approaches used for selective mutism. Based on nonparametric statistical tests of effect sizes, major findings include the following: treatment of selective mutism is more effective than no treatment; behaviorally oriented treatment approaches are more effective than no treatment; and no…

Phenomenology and treatment of selective mutism.

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Kumpulainen, Kirsti

2002-01-01

Selective mutism is a multidimensional childhood disorder in which, according to the most recent studies, biologically mediated temperament and anxiety components seem to play a major role. Several psychotherapy methods have been reported in case studies to be useful, but the disorder is commonly seen to be resistant to change, particularly in cases of long duration. Currently, behaviour modification and other cognitive methods, together with cooperation with the family and the school personnel, are recommended in the treatment of selective mutism. Selective serotonin reuptake inhibitors and selective monoamine oxidase inhibitors have also been reported to be helpful when treating children with selective mutism. At the moment, pharmacotherapy cannot be recommended as the treatment of first choice but if other methods of treatment are not helpful, medication can be included in the treatment scheme. Comprehensive evaluation and treatment of possible primary and comorbid problems that require treatment are also essential.

Two sets of twins with selective mutism: neuropsychological findings.

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Gray, Robert M; Jordan, Catherine M; Ziegler, Richard S; Livingston, Ronald B

2002-03-01

Neuropsychological data are reviewed from two sets of dizygotic twins presenting with selective mutism characterized by situation specific anxiety, extreme passive behavior, lack of responsivity, lack of peer interaction, and a chronic course of selective mutism. Both sets of twins had a history of prematurity and delayed speech development. One set of twins presented with normal intelligence and normal receptive language skills but with expressive language and oral motor sequencing difficulties. The second set of twins presented with Verbal IQ deficits and significant receptive and expressive language deficits. A summary of current conceptualizations regarding etiology and treatment of selective mutism is provided.

Group therapy for selective mutism - a parents' and children's treatment group.

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Sharkey, Louise; Mc Nicholas, Fiona; Barry, Edwina; Begley, Maire; Ahern, Sinead

2008-12-01

To evaluate the feasibility and effectiveness of group therapy for children with selective mutism and their parents. Five children (mean age 6.1 years) with a diagnosis of selective mutism were administered group therapy over an 8-week period. Parents simultaneously attended a second group, aimed at providing education and advice on managing selective mutism in everyday situations, and in the school environment. At post-treatment, all children increased their level of confident speaking in school, clinic and community settings. Parents indicated a reduction in their own anxiety levels, from pre- to post-treatment on self-rating scales. Findings support the feasibility and effectiveness of group therapy for children with selective mutism and their parents.

Selective Serotonin Reuptake Inhibitors for Treatment of Selective Mutism

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Mazlum Çöpür

2012-03-01

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

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

2014-11-01

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.

Selective Mutism: A Review of Etiology, Comorbidities, and Treatment

OpenAIRE

Wong, Priscilla

2010-01-01

Selective mutism is a rare and multidimensional childhood disorder that typically affects children entering school age. It is characterized by the persistent failure to speak in select social settings despite possessing the ability to speak and speak comfortably in more familiar settings. Many theories attempt to explain the etiology of selective mutism.

Behavior Observations for Linking Assessment to Treatment for Selective Mutism

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Shriver, Mark D.; Segool, Natasha; Gortmaker, Valerie

2011-01-01

Selective mutism is a childhood disorder that most school psychologists and educational providers will come across at least once in their careers. Selective mutism is associated with significant impairment in educational settings where speaking is necessary for academic and social skill development. Effective treatments for selective mutism…

Transient mutism and pathologic laughter in the course of cerebellitis.

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Dimova, Petia S; Bojinova, Veneta S; Milanov, Ivan G

2009-07-01

The phenomenon of cerebellar mutism with subsequent dysarthria is most commonly described as a part of posterior fossa syndrome after surgery for neoplasms in childhood. Pathologic laughter, on the other hand, is observed primarily in various neurologic diseases in adults. In the present case, a child manifested transient mutism and pathologic laughter during a severe cerebellitis. Headache, vertigo, and impaired consciousness developed during an acute respiratory infection. Thereafter, severe ataxia, mutism, and involuntary laughter became the main clinical features, as well as pyramidal signs. Magnetic resonance imaging revealed cerebellar swelling and T(2) hyperintensity. During steroid treatment, a gradual vanishing of the pathologic laughter and improvement of the motor and speech functions occurred. Recovery was slow and incomplete, and follow-up magnetic resonance imaging showed cerebellar atrophy. This case confirms that mutism is a rare, but possible, manifestation in acute parainfectious cerebellitis and provides a novel example of pathologic laughter during this disease in childhood.

Clinical distinctions between selective mutism and social phobia: an investigation of childhood psychopathology.

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Yeganeh, Robin; Beidel, Deborah C; Turner, Samuel M; Pina, Armando A; Silverman, Wendy K

2003-09-01

To investigate the hypothesis that children with selective mutism are more socially anxious than children with social anxiety disorder but who are not selectively mute. Twenty-three children with comorbid selective mutism and social phobia and 23 age-matched controls with social phobia alone and their parents participated in a comprehensive assessment of social anxiety and related aspects of psychopathology. The results do not uniformly support previous suggestions that children with selective mutism refuse speech because they are "frozen with fear." Although clinician and observer ratings for children with selective mutism revealed higher ratings of social distress than for children with social phobia alone, self-report data do not support this conclusion. Furthermore, although there were no group differences on measures of trait anxiety, general fears, or scores on the Child Behavior Checklist broadband Internalizing or Externalizing scales, children with selective mutism scored higher than children with social phobia alone on the Child Behavior Checklist Delinquency subscale, suggesting the presence of a broader clinical syndrome. It remains unclear whether children with selective mutism have extreme levels of social anxiety. Potential areas that might shed further light on this interesting disorder are discussed.

The Development and Psychometric Properties of the Selective Mutism Questionnaire

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Bergman, R. Lindsey; Keller, Melody L.; Piacentini, John; Bergman, Andrea J.

2008-01-01

Research on selective mutism (SM) has been limited by the absence of standardized, psychometrically sound assessment measures. The purpose of our investigation was to present two studies that examined the factor structure and initial reliability and validity of the Selective Mutism Questionnaire (SMQ), a 17-item parent report measure of failure to…

Selective Mutism: Phenomenological Characteristics.

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Ford, Mary Ann; Sladeczek, Ingrid E.; Carlson, John; Kratochwill, Thomas R.

1998-01-01

To explore factors related to selective mutism (SM), a survey of persons (N=153, including 135 children) with SM was undertaken. Three theoretical assumptions are supported: (1) variant talking behaviors prior to identification of SM; (2) link between SM and social anxiety; (3) potential link between temperament and SM. (EMK)

Selective Mutism: A Three-Tiered Approach to Prevention and Intervention

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Busse, R. T.; Downey, Jenna

2011-01-01

Selective mutism is a rare anxiety disorder that prevents a child from speaking at school or other community settings, and can be detrimental to a child's social development. School psychologists can play an important role in the prevention and treatment of selective mutism. As an advocate for students, school psychologists can work with teachers,…

Die terapeutiese gebruik van die scenotoets met verwysing na selektiewe mutisme

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Adre Nieuwoudt

2002-11-01

Full Text Available There is a paucity of applied research and available literature about selective mutism. Opsomming Daar is min toegepaste navorsing en beskikbare literatuur oor selektiewe mutisme. *Please note: This is a reduced version of the abstract. Please refer to PDF for full text.

Augmented Self-Modeling as a Treatment for Children with Selective Mutism.

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Kehle, Thomas J.; Madaus, Melissa R.; Baratta, Victoria S.; Bray, Melissa A.

1998-01-01

Describes the treatment of three children experiencing selective mutism. The procedure utilized incorporated self-modeling, mystery motivators, self-reinforcement, stimulus fading, spacing, and antidepressant medication. All three children evidenced a complete cessation of selective mutism and maintained their treatment gains at follow-up.…

Selective Mutism: Causes and Interventions.

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Hultquist, Alan M.

1995-01-01

This article reviews the literature regarding the diagnostic criteria, causes, assessment, and treatment of selective mutism in school-age children. The most successful treatments have included various forms or combinations of behavior modification, though these may not address the underlying problem. (Author/DB)

Cerebellar mutism: review of the literature

DEFF Research Database (Denmark)

Gudrunardottir, Thora; Sehested, Astrid; Juhler, Marianne

2011-01-01

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....

The Sounds of Silence: Language, Cognition, and Anxiety in Selective Mutism

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Manassis, Katharina; Tannock, Rosemary; Garland, E. Jane; Minde, Klaus; McInnes, Alison; Clark, Sandra

2007-01-01

Objectives: To determine whether oral language, working memory, and social anxiety differentiate children with selective mutism (SM), children with anxiety disorders (ANX), and normal controls (NCs) and explore predictors of mutism severity. Method: Children ages 6 to 10 years with SM (n = 44) were compared with children with ANX (n = 28) and NCs…

Arctic, Antarctic, and temperate green algae Zygnema spp. under UV-B stress: vegetative cells perform better than pre-akinetes.

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Holzinger, Andreas; Albert, Andreas; Aigner, Siegfried; Uhl, Jenny; Schmitt-Kopplin, Philippe; Trumhová, Kateřina; Pichrtová, Martina

2018-02-22

Species of Zygnema form macroscopically visible mats in polar and temperate terrestrial habitats, where they are exposed to environmental stresses. Three previously characterized isolates (Arctic Zygnema sp. B, Antarctic Zygnema sp. C, and temperate Zygnema sp. S) were tested for their tolerance to experimental UV radiation. Samples of young vegetative cells (1 month old) and pre-akinetes (6 months old) were exposed to photosynthetically active radiation (PAR, 400-700 nm, 400 μmol photons m -2  s -1 ) in combination with experimental UV-A (315-400 nm, 5.7 W m -2 , no UV-B), designated as PA, or UV-A (10.1 W m -2 ) + UV-B (280-315 nm, 1.0 W m -2 ), designated as PAB. The experimental period lasted for 74 h; the radiation period was 16 h PAR/UV-A per day, or with additional UV-B for 14 h per day. The effective quantum yield, generally lower in pre-akinetes, was mostly reduced during the UV treatment, and recovery was significantly higher in young vegetative cells vs. pre-akinetes during the experiment. Analysis of the deepoxidation state of the xanthophyll-cycle pigments revealed a statistically significant (p UV-absorbing phenolic compounds was significantly higher (p UV-B stress than pre-akinetes.

Pediatric selective mutism therapy: a randomized controlled trial.

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Esposito, Maria; Gimigliano, Francesca; Barillari, Maria R; Precenzano, Francesco; Ruberto, Maria; Sepe, Joseph; Barillari, Umberto; Gimigliano, Raffaele; Militerni, Roberto; Messina, Giovanni; Carotenuto, Marco

2017-10-01

Selective mutism (SM) is a rare disease in children coded by DSM-5 as an anxiety disorder. Despite the disabling nature of the disease, there is still no specific treatment. The aims of this study were to verify the efficacy of six-month standard psychomotor treatment and the positive changes in lifestyle, in a population of children affected by SM. Randomized controlled trial registered in the European Clinical Trials Registry (EuDract 2015-001161-36). University third level Centre (Child and Adolescent Neuropsychiatry Clinic). Study population was composed by 67 children in group A (psychomotricity treatment) (35 M, mean age 7.84±1.15) and 71 children in group B (behavioral and educational counseling) (37 M, mean age 7.75±1.36). Psychomotor treatment was administered by trained child therapists in residential settings three times per week. Each child was treated for the whole period by the same therapist and all the therapists shared the same protocol. The standard psychomotor session length is of 45 minutes. At T0 and after 6 months (T1) of treatments, patients underwent a behavioral and SM severity assessment. To verify the effects of the psychomotor management, the Child Behavior Checklist questionnaire (CBCL) and Selective Mutism Questionnaire (SMQ) were administered to the parents. After 6 months of psychomotor treatment SM children showed a significant reduction among CBCL scores such as in social relations, anxious/depressed, social problems and total problems (Pselective mutism, even if further studies are needed. The present study identifies in psychomotricity a safe and efficacy therapy for pediatric selective mutism.

Mutism as the presenting symptom: three case reports and selective review of literature.

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Aggarwal, Ashish; Sharma, Dinesh Dutt; Kumar, Ramesh; Sharma, Ravi C

2010-01-01

Mutism, defined as an inability or unwillingness to speak, resulting in an absence or marked paucity of verbal output, is a common clinical symptom seen in psychiatric as well as neurology outpatient department. It rarely presents as an isolated disability and often occurs in association with other disturbances in behavior, thought processes, affect, or level of consciousness. It is often a focus of clinical attention, both for the physician and the relatives. Mutism occurs in a number of conditions, both functional and organic, and a proper diagnosis is important for the management. We hereby present three cases, who presented with mutism as the presenting symptom and the differential diagnosis and management issues related to these cases are discussed. The authors also selectively reviewed the literature on mutism, including psychiatric, neurologic, toxic-metabolic, and drug-induced causes.

Too Anxious to Speak? The Implications of Current Research into Selective Mutism for Educational Psychology Practice

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Cleave, Hayley

2009-01-01

Selective Mutism is a low incidence disorder but has considerable impact on the school system when it occurs. Over the last decade several research articles have been published which have challenged the understanding of the aetiology of Selective Mutism. Current perceptions about the aetiology of Selective Mutism are considered in order to inform…

An Assessment Protocol for Selective Mutism: Analogue Assessment Using Parents as Facilitators.

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Schill, Melissa T.; And Others

1996-01-01

Assesses protocol for conducting a functional analysis of maintaining variables for children with selective mutism. A parent was trained in and later applied various behavior strategies designed to increase speech in an eight-year-old girl with selective mutism. Parent and child ratings of treatment were positive. Presents implications for future…

Treating Selective Mutism Using Modular CBT for Child Anxiety: A Case Study

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Reuther, Erin T.; Davis, Thompson E., III; Moree, Brittany N.; Matson, Johnny L.

2011-01-01

Selective mutism is a rare, debilitating condition usually seen in children. Unfortunately, there is little research examining effective treatments for this disorder, and designing an evidence-based treatment plan can be difficult. This case study presents the evidence-based treatment of an 8-year-old Caucasian boy with selective mutism using an…

Factors Affecting the Germination of Akinetes of Nodularia spumigena (Cyanobacteriaceae)

OpenAIRE

Huber, Ann L.

1985-01-01

Nutritional and physical factors which influence the germination of akinetes of Nodularia spumigena (Cyanobacteriaceae) were examined. Low concentrations of phosphorus (45 μM, inhibited germination. Salinities of >20‰ were inhibitory to germination. Optimum temperatures were 22°C or greater. Germination did not take place in the dark, but only very low light intensities (0.5 microeinstein m−2 s−1) were necessary to initiate germination. Red light (620 to 665 nm) was required. More than 24 h o...

Elective Mutism Associated with Selective Inactivity.

Science.gov (United States)

Hill, Linda; Scull, John

1985-01-01

Effective treatment procedures for a nine-year-old boy with elective mutism and selective inactivity included increasing the frequency of situations in which he could already speak and decreasing the frequency of those in which he seldom spoke (specifically coercive situations). (CL)

A highly asynchronous developmental program triggered during germination of dormant akinetes of filamentous diazotrophic cyanobacteria

Czech Academy of Sciences Publication Activity Database

Perez, Rebeca; Wörmer, L.; Sass, P.; Maldener, I.

2018-01-01

Roč. 94, č. 1 (2018), č. článku fix131. ISSN 0168-6496 Institutional support: RVO:61388971 Keywords : akinetes * germination * heterocyst Subject RIV: EE - Microbiology, Virology OBOR OECD: Microbiology Impact factor: 3.720, year: 2016

Fluorodeoxyglucose positron emission tomography/computed tomography findings in a patient with cerebellar mutism after operation in posterior fossa

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Gonca Kara Gedik

2017-03-01

Full Text Available Cerebellar mutism is a transient period of speechlessness that evolves after posterior fossa surgery in children. Although direct cerebellar and brain stem injury and supratentorial dysfunction have been implicated in the mediation of mutism, the pathophysiological mechanisms involved in the evolution of this kind of mutism remain unclear. Magnetic resonance imaging revealed dentatothalamocortical tract injuries and single photon emission computed tomography showed cerebellar and cerebral hypoperfusion in patients with cerebellar mutism. However, findings with 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT in this group of patients have not been documented previously. In this clinical case, we report a patient who experienced cerebellar mutism after undergoing a posterior fossa surgery. Right cerebellar and left frontal lobe hypometabolism was shown using FDG PET/CT. The FDG metabolism of both the cerebellum and the frontal lobe returned to normal levels after the resolution of the mutism symptoms.

Silent Suffering: Children with Selective Mutism

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Camposano, Lisa

2011-01-01

Despite increasing awareness, the childhood disorder of selective mutism is under-researched and commonly misdiagnosed. The purpose of this article is to highlight current issues related to this disorder as well as describe various treatment approaches including behavioral, cognitive-behavioral, psychodynamic, family, and pharmacological…

Including Children with Selective Mutism in Mainstream Schools and Kindergartens: Problems and Possibilities

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Omdal, Heidi

2008-01-01

There is little research on inclusion of children with selective mutism in school/kindergarten. Moreover, few studies have tried to understand selectively mute children's interactions in the natural surroundings of their home and school/kindergarten. Five children meeting the DSM-IV criteria for selective mutism were video-observed in social…

Selective Mutism: Definition, Issues, and Treatment.

Science.gov (United States)

Brigham, Frederick J.; Cole, Jane E.

This paper reviews definitions and issues in selective mutism in children and summarizes results of interventions conducted and published since 1982. Definitions and diagnostic criteria of the American Psychiatric Association's "Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) (1994)" and the World Health Organization's…

Selective Mutism: Treating the Silent Child

Science.gov (United States)

Shott, Elizabeth F.; Warren, Mary Ellen

2011-01-01

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…

Treatment of selective mutism: focus on selective serotonin reuptake inhibitors.

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Kaakeh, Yaman; Stumpf, Janice L

2008-02-01

Abstract Selective mutism is a pediatric psychiatric disorder that occurs when a child consistently fails to speak in specific situations in which speaking is expected, such as at school and social gatherings, but speaks appropriately in other settings. Selective mutism often is diagnosed when a child starts school and does not talk to teachers or peers, but talks to family members at home; the condition is frequently accompanied by anxiety and shyness. Although the underlying etiology of the condition remains unclear, psychotherapy is the preferred initial treatment, with the support of parents and teachers. If the child does not respond to psychotherapy, addition of pharmacologic treatment should be considered, depending on the severity of symptoms and presence of other illnesses. Although data are limited to case reports and trials with small patient populations and short follow-up periods, some patients with selective mutism respond to therapy with selective serotonin reuptake inhibitors (SSRIs). Fluoxetine is the most studied SSRI as treatment for the condition, although further investigation is required to determine the optimal dosage and duration of therapy.

[Outpatient treatment of selective mutism: long-standing selective mutism in a 17-year-old male].

Science.gov (United States)

Herdener-Pinnekamp, Katharina; Gundelfinger, Ronnie; Steinhausen, Hans-Christoph

2010-01-01

The present case report describes the successful treatment of a 17 year old male adolescent suffering for 10 years from selective mutism. Following a summary review of recent publications on therapy approaches, the report describes the treatment concept in the present case, including detailed assessment of co-morbid disorders, motivation for change, behaviour therapy with supporting drug intervention, and intensive co-operation with parents and other caretakers.

Female monozygotic twins with selective mutism--a case report.

Science.gov (United States)

Sharkey, L; Mc Nicholas, F

2006-04-01

Selective mutism is a rare social anxiety disorder characterized by a total lack of speech in certain specific situations despite the ability to speak in others. Both genetic and psychosocial factors are thought to be involved in its presentation, persistence, and response to treatment. This case report describes a case of young female monozygotic twins who presented with selective mutism and their treatment spanning a 2-year period. It highlights the strong genetic association along with environmental factors such as social isolation and consequences of maternal social phobia, all contributing to treatment resistance, despite an intensive multimodal biopsychosocial approach. General issues related to the difficulties in treating monozygotic twins are also addressed.

Creutzfeldt-Jakob Disease: Analysis of Four Cases

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Ali Al Balushi

2016-08-01

Full Text Available Background: Creutzfeldt-Jakob disease (CJD is a rare, rapidly progressive neurodegenerative disease that almost always results in death in under a year from onset of symptoms. Here, we report four cases of CJD with different clinical presentations diagnosed at our institution over two-year period. Cases: The first patient is an 82-year-old woman who presented with depression, cognitive decline and word-finding difficulty over 4 weeks. The patient deteriorated neurologically to akinetic mutism and death within 6 weeks of presentation. The second patient is a 54-year-old woman with liver cirrhosis who presented with confusion, ataxia and multiple falls over 4 weeks. She was treated initially for hepatic encephalopathy, but continued to progress to mutism, startle myoclonus and obtundation. Death occurred within 4 weeks of presentation. The third patient is a 58-year-old woman who presented with an 8-week history of confusion, urinary incontinence, Parkinsonism, ataxia and myoclonus. Death occurred within 2 months from presentation. The fourth patient is a 67-year-old man who presented with a 6-week history of headache, blurred vision, ataxia and personality change and progressed to confusion, myoclonus, akinetic mutism and obtundation. Death occurred within 3 weeks from presentation. Conclusion: These 4 cases highlight the varied possible clinical presentations of CJD and demonstrate the importance of considering CJD in patients with atypical presentations of rapidly progressive cognitive decline. To diagnose CJD, brain biopsy remains the gold standard. However, the presence of CSF protein 14-3-3, typical MRI findings and suggestive EEG abnormalities all support the diagnosis.

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

Energy Technology Data Exchange (ETDEWEB)

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)

2015-12-31

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.

Functional Assessment-Based Intervention for Selective Mutism

Science.gov (United States)

Kern, Lee; Starosta, Kristin M.; Bambara, Linda M.; Cook, Clayton R.; Gresham, Frank R.

2007-01-01

The process of functional assessment has emerged as an essential component for intervention development. Applications across divergent types of problem behavior, however, remain limited. This study evaluated the applicability of this promising approach to students with selective mutism. Two middle school students served as participants. The…

Selective Mutism in Elementary School: Multidisciplinary Interventions.

Science.gov (United States)

Giddan, Jane J.; And Others

1997-01-01

Presents the symptoms of selective mutism and historical background for treatment. It provides a case study which illustrates successful multidisciplinary treatment outcomes for a child who was selectively mute. Issues relevant to speech-language pathologists working with elementary school children are discussed and treatment guidelines provided.…

The Selective Mutism Questionnaire: Measurement Structure and Validity

Science.gov (United States)

Letamendi, Andrea M.; Chavira, Denise A.; Hitchcock, Carla A.; Roesch, Scott C.; Shipon-Blum, Elisa; Stein, Murray B.; Roesch, Scott C.

2010-01-01

Objective To evaluate the factor structure, reliability, and validity of the 17-item Selective Mutism Questionnaire. Method Diagnostic interviews were administered via telephone to 102 parents of children identified with selective mutism (SM) and 43 parents of children without SM from varying U.S. geographic regions. Children were between the ages of 3 and 11 inclusive and comprised 58% girls and 42% boys. SM diagnoses were determined using the Anxiety Disorders Interview Schedule for Children - Parent Version (ADIS-C/P); SM severity was assessed using the 17-item Selective Mutism Questionnaire (SMQ); and behavioral and affective symptoms were assessed using the Child Behavior Checklist (CBCL). An exploratory factor analysis (EFA) was conducted to investigate the dimensionality of the SMQ and a modified parallel analysis procedure was used to confirm EFA results. Internal consistency, construct validity, and incremental validity were also examined. Results The EFA yielded a 13-item solution consisting of three factors: a) Social Situations Outside of School, b) School Situations, and c) Home and Family Situations. Internal consistency of SMQ factors and total scale ranged from moderate to high. Convergent and incremental validity were also well supported. Conclusions Measure structure findings are consistent with the 3-factor solution found in a previous psychometric evaluation of the SMQ. Results also suggest that the SMQ provides useful and unique information in the prediction of SM phenomenon beyond other child anxiety measures. PMID:18698268

JPH3 Repeat Expansions Cause a Progressive Akinetic-Rigid Syndrome with Severe Dementia and Putaminal Rim in a Five-Generation African-American Family

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Schneider, Susanne A.; Marshall, Kate E.; Xiao, Jianfeng; LeDoux, Mark S.

2012-01-01

We report the clinical, neuropsychological, genetic and radiological features of a large five-generation African-American kindred from the southern United States presenting with a progressive akinetic-rigid syndrome and severe dementia, but clinically insignificant chorea, due to mutations in JPH3. Overt disease onset was in the mid-twenties to late thirties with cognitive decline, REM sleep disturbance or psychiatric features, followed by development of a levodopa-unresponsive akinetic-rigid motor syndrome. Dystonia and myoclonus were present in some subjects. A bedridden, non-verbal severely akinetic-rigid state developed within 10 to 15 years after onset. CTG repeat expansions ranged from 47 to 53. Imaging revealed generalized cerebral atrophy with severe striatal involvement and putaminal rim hyperintensity. Analysis of our kindred indicates that JPH3 mutations should be considered in the differential diagnosis of early-onset dementia and hypokinetic-rigid syndromes in individuals of African descent. Moreover, chorea may not be overtly manifest at presentation or during significant parts of the disease course. PMID:22447335

Treating Youths with Selective Mutism with an Alternating Design of Exposure-Based Practice and Contingency Management

Science.gov (United States)

Vecchio, Jennifer; Kearney, Christopher A.

2009-01-01

Selective mutism is a severe childhood disorder involving failure to speak in public situations in which speaking is expected. The present study examined 9 youths with selective mutism treated with child-focused, exposure-based practices and parent-focused contingency management via an alternating treatments design. Broadband measures of…

Anxiety and oppositional behavior profiles among youth with selective mutism.

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Diliberto, Rachele A; Kearney, Christopher A

2016-01-01

Selective mutism (SM) is a debilitating condition in which a child does not speak in social situations where speech is expected. The clinical conceptualization of SM has been debated historically, with evidence pointing partly to anxious and oppositional behavior profiles. Behavioral characteristics were examined in a clinical sample of 57 youth formally diagnosed with selective mutism. Parents rated children across internalizing and externalizing behaviors on the Child Behavior Checklist. Eighteen highly rated items were subjected to exploratory and then confirmatory factor analysis. Anxiety and oppositional behavior factors were derived. The anxious behavior profile was associated with social anxiety disorder symptoms, social problems, and aggressive behaviors but not oppositional defiant disorder symptoms. The oppositional behavior profile was associated with aggressive behaviors, oppositional defiant disorder symptoms, social problems, and inversely to social anxiety disorder symptoms. Results are consistent with emerging research regarding subgroups of children with SM. Behavior profiles are discussed as well with respect to assessment and treatment implications. Readers will learn about the nature of children with selective mutism as well as behaviors that differentiate anxious and oppositional behavior profiles. Items that comprise anxious and oppositional behavior profiles are presented. These item profiles may have ramifications for assessment and treatment. Copyright © 2015 Elsevier Inc. All rights reserved.

Multimethod Behavioral Treatment of Long-Term Selective Mutism.

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Watson, T. Steuart; Kramer, Jack J.

1992-01-01

Conducted single-subject, experimental research to examine efficacy of treating severe, long-term selective mutism in nine-year-old male using shaping, multiple reinforcers, natural consequences, stimulus fading, and mild aversives. Implemented different treatment regimens in home and school environments. Home intervention resulted in increase in…

Selective mutism: an update and suggestions for future research.

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Scott, Samantha; Beidel, Deborah C

2011-08-01

Speculation continues regarding the accurate classification of selective mutism and potential etiologic factors. Current research has shed some light on several factors that may predispose some children to this disorder, but conclusions are difficult to draw due to reliance on subjective measures, few comparison groups, and/or limited theoretical grounding. This article provides an update on recent efforts to elucidate the etiologic pathways of selective mutism and on the current debate regarding its strong overlap with anxiety disorders, most notably social phobia. An additional attempt is made to examine findings based on a developmental perspective that accounts for multiple pathways, context, and the developmental stage of the child. Emotion regulation theory is offered as a potential factor in why some children may be more vulnerable to the etiologic factors described. Suggestions for future research are offered based on this integration of information.

Social phobia and selective mutism.

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Keeton, Courtney P; Crosby Budinger, Meghan

2012-07-01

Social phobia (SOP) and selective mutism (SM) are related anxiety disorders characterized by distress and dysfunction in social situations. SOP typically onsets in adolescence and affects about 8% of the general population, whereas SM onsets before age 5 and is prevalent in up to 2% of youth. Prognosis includes a chronic course that confers risk for other disorders or ongoing social disability, but more favorable outcomes may be associated with young age and low symptom severity. SOP treatments are relatively more established, whereas dissemination of promising and innovative SM-treatment strategies is needed.

Integrated Behavior Therapy for Selective Mutism: a randomized controlled pilot study.

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Bergman, R Lindsey; Gonzalez, Araceli; Piacentini, John; Keller, Melody L

2013-10-01

To evaluate the feasibility, acceptability, and preliminary efficacy of a novel behavioral intervention for reducing symptoms of selective mutism and increasing functional speech. A total of 21 children ages 4 to 8 with primary selective mutism were randomized to 24 weeks of Integrated Behavior Therapy for Selective Mutism (IBTSM) or a 12-week Waitlist control. Clinical outcomes were assessed using blind independent evaluators, parent-, and teacher-report, and an objective behavioral measure. Treatment recipients completed a three-month follow-up to assess durability of treatment gains. Data indicated increased functional speaking behavior post-treatment as rated by parents and teachers, with a high rate of treatment responders as rated by blind independent evaluators (75%). Conversely, children in the Waitlist comparison group did not experience significant improvements in speaking behaviors. Children who received IBTSM also demonstrated significant improvements in number of words spoken at school compared to baseline, however, significant group differences did not emerge. Treatment recipients also experienced significant reductions in social anxiety per parent, but not teacher, report. Clinical gains were maintained over 3 month follow-up. IBTSM appears to be a promising new intervention that is efficacious in increasing functional speaking behaviors, feasible, and acceptable to parents and teachers. Copyright © 2013 Elsevier Ltd. All rights reserved.

Differential Diagnosis of Selective Mutism in Bilingual Children

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Toppelberg, Claudio O.; Tabors, Patton; Coggins, Alissa; Lum, Kirk; Burger, Claudia

2005-01-01

Early diagnosis of selective mutism (SM) is an important concern. SM prevalence is higher than initially thought and at least three times higher in immigrant language minority children. Although the DSM-IV precludes diagnosing SM in immigrant children with limited language proficiency (as children acquiring a second language may normally undergo a…

Selective Mutism and Social Anxiety Disorder: All in the Family?

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Chavira, Denise A.; Shipon-Blum, Elisa; Hitchcock, Carla; Cohan, Sharon; Stein, Murray B.

2007-01-01

The relationship between a history of lifetime psychiatric disorders of parents with selective mutism (SM) in their children is examined. The results support earlier findings of a familial relationship between generalized social phobia and SM.

A Controlled Single-Case Treatment of Severe Long-Term Selective Mutism in a Child with Mental Retardation

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Facon, Bruno; Sahiri, Safia; Riviere, Vinca

2008-01-01

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…

Children with autism spectrum disorders and selective mutism

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Steffenburg H

2018-05-01

Full Text Available Hanna Steffenburg, Suzanne Steffenburg, Christopher Gillberg, Eva Billstedt Gillberg Neuropsychiatry Centre, Institute of Neuroscience and Physiology, University of Gothenburg, Sahlgrenska Academy, Gothenburg, Sweden Background: It has been suggested that autism spectrum disorder (ASD might be a “comorbid” condition in selective mutism (SM. Methods: In this retrospective study, we examined medical records of children with SM diagnosis (n=97 at a medical center specializing in assessment of ASD. Results: Mean age for onset of SM symptoms was 4.5 years and mean age at SM diagnosis was 8.8 years. SM was more common among girls (boy:girl ratio=2.7:1. We found that 63% of the study group had an ASD (no gender difference. The SM group with combined ASD had later onset of symptoms, higher age at diagnosis, more often a history of speech delay, and a higher proportion of borderline IQ or intellectual disability.Conclusion: The results highlight the risk of overlap between ASD and SM. Keywords: selective mutism, autism spectrum disorders, Asperger syndrome, autistic disorder

Case Report: Evaluation strategies and cognitive intervention: the case of a monovular twin child affected by selective mutism.

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Capobianco, Micaela; Cerniglia, Luca

2018-01-01

The present work describes the assessment process, evaluation strategies, and cognitive intervention on a 9 years old child with selective mutism (SM), a monovular twin of a child also affected by mutism. Currently, the cognitive behavioral multimodal treatment seems the most effective therapeutic approach for children diagnosed with selective mutism (Capobianco & Cerniglia, 2018). The illustrated case confirms the role of biological factors involved in mutacic disorder but also highlights the importance of environmental influences in the maintenance of the disorder with respect to relational and contextual dynamics (e.g. complicity between sisters, family relationships). The article discusses furthermore the importance of an early diagnosis as a predictor of positive treatment outcomes.

Selective Mutism: Practice and Intervention Strategies for Children

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

2012-01-01

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…

Subacute sclerosing panencephalitis

International Nuclear Information System (INIS)

Modi, G.; Bill, P.; Campbell, H.

1989-01-01

A 19-year-old female patient presented in an acute state of akinetic mutism. Serological analysis of serum and cerebrospinal fluid demonstrated the presence of antibodies to measles virus. CT scan carried out during this acute phase of relapse demonstrated white matter enhancement affecting the cortical white matter of the frontal lobes and corpus callosum. These features indicate that active demyelination occurs during acute relapse in subacute sclerosing panencephalitis (SSPE) and suggest that immunotherapy should be considered during this acute phase. (orig.)

Augmented Self-Modeling as an Intervention for Selective Mutism

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Kehle, Thomas J.; Bray, Melissa A.; Byer-Alcorace, Gabriel F.; Theodore, Lea A.; Kovac, Lisa M.

2012-01-01

Selective mutism is a rare disorder that is difficult to treat. It is often associated with oppositional defiant behavior, particularly in the home setting, social phobia, and, at times, autism spectrum disorder characteristics. The augmented self-modeling treatment has been relatively successful in promoting rapid diminishment of selective mutism…

Reduced auditory efferent activity in childhood selective mutism.

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Bar-Haim, Yair; Henkin, Yael; Ari-Even-Roth, Daphne; Tetin-Schneider, Simona; Hildesheimer, Minka; Muchnik, Chava

2004-06-01

Selective mutism is a psychiatric disorder of childhood characterized by consistent inability to speak in specific situations despite the ability to speak normally in others. The objective of this study was to test whether reduced auditory efferent activity, which may have direct bearings on speaking behavior, is compromised in selectively mute children. Participants were 16 children with selective mutism and 16 normally developing control children matched for age and gender. All children were tested for pure-tone audiometry, speech reception thresholds, speech discrimination, middle-ear acoustic reflex thresholds and decay function, transient evoked otoacoustic emission, suppression of transient evoked otoacoustic emission, and auditory brainstem response. Compared with control children, selectively mute children displayed specific deficiencies in auditory efferent activity. These aberrations in efferent activity appear along with normal pure-tone and speech audiometry and normal brainstem transmission as indicated by auditory brainstem response latencies. The diminished auditory efferent activity detected in some children with SM may result in desensitization of their auditory pathways by self-vocalization and in reduced control of masking and distortion of incoming speech sounds. These children may gradually learn to restrict vocalization to the minimal amount possible in contexts that require complex auditory processing.

Diffusion MR imaging in sporadic Creutzfeldt-Jakob disease

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Burcak Cakir Pekoz

2014-08-01

Full Text Available Creutzfeldt-Jakob disease (CJD is a rare dementing disease and is thought to caused by a prion. It is characterized by rapidly progressive dementia, ataxia, myoclonus, akinetic mutism and eventual death. Brain biopsy or autopsy is required for a definitive diagnosis of CJD. Diffusion-weighted imaging became an important tool for early diagnosis of CJD because of the high sensitivity. We present 59-year-old female patient diagnosed as sporadic CJD with typical MR imagings. [Cukurova Med J 2014; 39(4.000: 880-883

Silent suffering: understanding and treating children with selective mutism.

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Manassis, Katharina

2009-02-01

Children with selective mutism (SM) restrict speech in some social environments, often resulting in substantial academic and social impairment. Although SM is considered rare, one or more children with SM can be found in most elementary schools. Assessment is performed to confirm the diagnosis, rule out psychological and medical factors that may account for the mutism, ascertain comorbid and exacerbating conditions needing treatment, and develop an intervention plan. Interventions are often multidisciplinary and focus on decreasing anxiety, increasing social speech and ameliorating SM-related impairment. Research is limited, but symptomatic improvement has been demonstrated with behavioral interventions and multimodal treatments that include school and family participation, as well as behavioral methods. Selective serotonin-reuptake inhibitors, especially fluoxetine, have also been found to be efficacious and merit consideration in severe cases. Persistence of some SM or anxiety symptoms despite treatment is common. Further development of treatments targeting specific etiological factors, comparative treatment studies and determination of optimal involvement of families and schools in treatment are needed to improve outcomes for children with SM.

Language and Academic Abilities in Children with Selective Mutism

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Nowakowski, Matilda E.; Cunningham, Charles E.; McHolm, Angela E.; Evans, Mary Ann; Edison, Shannon; St. Pierre, Jeff; Boyle, Michael H.; Schmidt, Louis A.

2009-01-01

We examined receptive language and academic abilities in children with selective mutism (SM; n = 30; M age = 8.8 years), anxiety disorders (n = 46; M age = 9.3 years), and community controls (n = 27; M age = 7.8 years). Receptive language and academic abilities were assessed using standardized tests completed in the laboratory. We found a…

Narrative Skills in Children with Selective Mutism: An Exploratory Study

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McInnes, Alison; Fung, Daniel; Manassis, Katharina; Fiksenbaum, Lisa; Tannock, Rosemary

2004-01-01

Selective mutism (SM) is a rare and complex disorder associated with anxiety symptoms and speech-language deficits; however, the nature of these language deficits has not been studied systematically. A novel cross-disciplinary assessment protocol was used to assess anxiety and nonverbal cognitive, receptive language, and expressive narrative…

Factors influencing the survival period in Japanese patients with sporadic Creutzfeldt-Jakob disease.

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Iwasaki, Yasushi; Akagi, Akio; Mimuro, Maya; Kitamoto, Tetsuyuki; Yoshida, Mari

2015-10-15

Although Japanese cases of sporadic Creutzfeldt-Jakob disease (sCJD) generally involve longer survival periods compared to those from other countries, details regarding the factors influencing survival are unclear. To determine the influence of certain factors on survival, we retrospectively assessed 51 Japanese MM1-type sCJD patients with respect to background, clinical course, and disease management. No significant differences were found between men and women, tracheotomy and nontracheotomy patients, or patients treated in public and other types of hospitals. Although the survival period of tube-fed patients was significantly longer than that of patients who were not tube fed, survival of patients fed via a nasal tube did not differ significantly from that of gastrostomy-fed patients. The proportion of tube-fed patients was 68.6% (35/51). Disease duration was not significantly associated with age or year of onset. However, it was associated with time from onset to first recognition of myoclonus, first recognition of periodic sharp-wave complexes on electroencephalogram, and progression to the akinetic mutism state. Mechanical ventilation was not performed for any patient. Because the total disease duration increased in cases with a slowly progressive clinical course as a natural outcome, we concluded that the most crucial factor contributing to the prolonged survival of Japanese sCJD patients was tube feeding once the akinetic mutism state had been reached. Copyright © 2015 Elsevier B.V. All rights reserved.

Overcoming Mutism in Adults with Learning Disabilities: A Case Study.

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Bell, Dorothy M.; Espie, Colin A.

2003-01-01

A woman with Down syndrome, who had shown selective mutism for more than 14 years, successfully participated in a program designed to reinforce communication and gradually increase the number of words spoken to one person and then to others. Nonaversive behavior methods were used and response initiative procedures were developed. (Contains…

Significance of cultural beliefs in presentation of psychiatric illness: a case report of selective mutism in a man from Nepal.

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Babikian, Sarkis; Emerson, Lyndal; Wynn, Gary H

2007-11-01

A 22-year-old active duty E1 Nepalese male who recently emigrated from Nepal suddenly exhibited strange behaviors and mutism during Advanced Individual Training. After receiving care from a hospital near his unit, he was transferred to Walter Reed Army Medical Center Inpatient Psychiatry for further evaluation and treatment. Although he was admitted with a diagnosis of psychosis not otherwise specified (NOS), after consideration of cultural factors and by ruling out concurrent thought disorder, a diagnosis of selective mutism was made. To our knowledge this is the first reported case of selective mutism in a soldier. This case serves as a reminder of the need for cultural awareness during psychological evaluation, diagnosis, and treatment of patients.

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.

2008-01-01

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

A Long-Term Outcome Study of Selective Mutism in Childhood

Science.gov (United States)

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

2006-01-01

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…

Assessment and Treatment of Selective Mutism with English Language Learners

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

2015-01-01

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…

A case of selective mutism in an 8-year-old girl with thalassaemia major after bone marrow transplantation.

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Plener, P L; Gatz, S A; Schuetz, C; Ludolph, A G; Kölch, M

2012-01-01

Selective mutism is rare with a prevalence below 1% in the general population, but a higher prevalence in populations at risk (children with speech retardation, migration). Evidence for treatment strategies is hardly available. This case report provides information on the treatment of selective mutism in an 8-year-old girl with preexisting thalassaemia major. As medications she received penicillin prophylaxis (500000 IE/d) and deferasirox (Exjade; 20-25mg/kg/d), an iron chelator. The preexisting somatic disease and treatment complicated the treatment, as there are no data about pharmacological combination therapy. Psychotherapy in day treatment, supported by the use of the SSRI fluoxetine (10 mg), led to a decrease in the selective mutism score from 33 to 12 points, GAF improved by 21 points. Mean levels of fluoxetine plus norfluoxetine were 287.8 ng/ml without significant level fluctuations. © Georg Thieme Verlag KG Stuttgart · New York.

P50 Suppression in Children with Selective Mutism: A Preliminary Report

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Henkin, Yael; Feinholz, Maya; Arie, Miri; Bar-Haim, Yair

2010-01-01

Evidence suggests that children with selective mutism (SM) display significant aberrations in auditory efferent activity at the brainstem level that may underlie inefficient auditory processing during vocalization, and lead to speech avoidance. The objective of the present study was to explore auditory filtering processes at the cortical level in…

Selective Mutism in Immigrant Children: Cultural Considerations for Assessment and Intervention

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Thomson, Becky

2017-01-01

Selective mutism (SM) is a childhood anxiety disorder characterized by the persistent failure to speak in situations where speech is typically expected (e.g., school), despite speaking in other situations (e.g., home; American Psychiatric Association [APA], 2013). Immigrant children are more likely to be diagnosed with SM than the general…

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

Science.gov (United States)

O'Reilly, Mark; McNally, Deirdre; Sigafoos, Jeff; Lancioni, Giulio E.; Green, Vanessa; Edrisinha, Chaturi; Machalicek, Wendy; Sorrells, Audrey; Lang, Russell; Didden, Robert

2008-01-01

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 instruction from a therapist and was taught to…

Assessment and Treatment of Selective Mutism: Recommendations and a Case Study.

Science.gov (United States)

Sheridan, Susan M.; And Others

1995-01-01

Presents results of stimulus fading procedure used to treat a six-year-old girl with elective mutism. Presents assessment protocol that features procedures that can be used to render a diagnosis and assist in treatment planning. Results suggest stimulus fading of new persons into the school setting was effective in producing speech. (JBJ)

Meeting Educators Where They Are: Professional Development to Address Selective Mutism

Science.gov (United States)

Harwood, Debra; Bork, Po-Ling

2011-01-01

Children with selective mutism (SM) present unique challenges for teachers. Typically, children with SM have such an immense anxiety associated with being seen or heard speaking they fail to speak inside the classroom and particularly with teachers. This article reports on the effectiveness of a small-scale exploratory study involving 22…

Blindness and Selective Mutism: One Student's Response to Voice-Output Devices

Science.gov (United States)

Holley, Mary; Johnson, Ashli; Herzberg, Tina

2014-01-01

This case study was designed to measure the response of one student with blindness and selective mutism to the intervention of voice-output devices across two years and two different teachers in two instructional settings. Before the introduction of the voice output devices, the student did not choose to communicate using spoken language or…

The Silent Minority: Supporting Students with Selective Mutism Using Systemic Perspectives

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Lawrence, Zena

2017-01-01

Selective Mutism (SM) is an under-researched area of child development. While the incidence rate is low, the impact of this difficulty can be pervasive and can present as a significant risk for student mental health and wellbeing. The following article presents a case study focusing on parent-student intervention for a preadolescent male, using an…

Case Report: Evaluation strategies and cognitive intervention: the case of a monovular twin child affected by selective mutism [version 1; referees: 2 approved

Directory of Open Access Journals (Sweden)

Micaela Capobianco

2018-02-01

Full Text Available The present work describes the assessment process, evaluation strategies, and cognitive intervention on a 9 years old child with selective mutism (SM, a monovular twin of a child also affected by mutism. Currently, the cognitive behavioral multimodal treatment seems the most effective therapeutic approach for children diagnosed with selective mutism (Capobianco & Cerniglia, 2018. The illustrated case confirms the role of biological factors involved in mutacic disorder but also highlights the importance of environmental influences in the maintenance of the disorder with respect to relational and contextual dynamics (e.g. complicity between sisters, family relationships. The article discusses furthermore the importance of an early diagnosis as a predictor of positive treatment outcomes.

Sluggish vagal brake reactivity to physical exercise challenge in children with selective mutism.

Science.gov (United States)

Heilman, Keri J; Connolly, Sucheta D; Padilla, Wendy O; Wrzosek, Marika I; Graczyk, Patricia A; Porges, Stephen W

2012-02-01

Cardiovascular response patterns to laboratory-based social and physical exercise challenges were evaluated in 69 children and adolescents, 20 with selective mutism (SM), to identify possible neurophysiological mechanisms that may mediate the behavioral features of SM. Results suggest that SM is associated with a dampened response of the vagal brake to physical exercise that is manifested as reduced reactivity in heart rate and respiration. Polyvagal theory proposes that the regulation of the vagal brake is a neurophysiological component of an integrated social engagement system that includes the neural regulation of the laryngeal and pharyngeal muscles. Within this theoretical framework, sluggish vagal brake reactivity may parallel an inability to recruit efficiently the structures involved in speech. Thus, the findings suggest that dampened autonomic reactivity during mobilization behaviors may be a biomarker of SM that can be assessed independent of the social stimuli that elicit mutism.

A Selective Mutism Arising from First Language Attrition, Successfully Treated with Paroxetine-CBT Combination Treatment.

Science.gov (United States)

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

2015-10-01

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.

Unable to Speak: Selective Mutism in Youth.

Science.gov (United States)

Smith-Schrandt, Heather L; Ellington, Erin

2018-02-01

It is important for psychiatric nurses to be familiar with the clinical presentation and recommended treatment for selective mutism (SM), as it is a childhood anxiety disorder that is not commonly studied. This article provides a brief overview of its diagnostic criteria, prevalence, assessment, and history. Special attention is given to misconceptions regarding the disorder and differentiation of trauma and oppositional disorders. Two vignettes illustrate varied presentations of SM, with and without comorbid social phobia. Empirically supported behavioral and psychopharmacological treatment is outlined, and considerations for nursing are provided. [Journal of Psychosocial Nursing and Mental Health Services, 56(2), 14-18.]. Copyright 2018, SLACK Incorporated.

Suffering in silence: why a developmental psychopathology perspective on selective mutism is needed.

Science.gov (United States)

Cohan, Sharon L; Price, Joseph M; Stein, Murray B

2006-08-01

A developmental psychopathology perspective is offered in an effort to organize the existing literature regarding the etiology of selective mutism (SM), a relatively rare disorder in which a child consistently fails to speak in 1 or more social settings (e.g., school) despite speaking normally in other settings (e.g., home). Following a brief description of the history, prevalence, and course of the disorder, multiple pathways to the development of SM are discussed, with a focus on the various genetic, temperamental, psychological, and social/environmental systems that may be important in conceptualizing this unusual childhood disorder. The authors propose that SM develops due to a series of complex interactions among the various systems reviewed (e.g., a strong genetic loading for anxiety interacts with an existing communication disorder, resulting in heightened sensitivity to verbal interactions and mutism in some settings). Suggestions are provided for future longitudinal, twin/adoption, molecular genetic, and neuroimaging studies that would be particularly helpful in testing the pathways perspective on SM.

Refining the Classification of Children with Selective Mutism: A Latent Profile Analysis

Science.gov (United States)

Cohan, Sharon L.; Chavira, Denise A.; Shipon-Blum, Elisa; Hitchcock, Carla; Roesch, Scott C.; Stein, Murray B.

2008-01-01

The goal of this study was to develop an empirically derived classification system for selective mutism (SM) using parent-report measures of social anxiety, behavior problems, and communication delays. The sample consisted of parents of 130 children (ages 5-12) with SM. Results from latent profile analysis supported a 3-class solution made up of…

Akinetic-rigid and tremor-dominant Parkinson's disease patients show different patterns of intrinsic brain activity.

Science.gov (United States)

Zhang, Jiuquan; Wei, Luqing; Hu, Xiaofei; Xie, Bing; Zhang, Yanling; Wu, Guo-Rong; Wang, Jian

2015-01-01

Parkinson's disease (PD) is a surprisingly heterogeneous neurodegenerative disorder. It is well established that different subtypes of PD present with different clinical courses and prognoses. However, the neural mechanism underlying these disparate presentations is uncertain. Here we used resting-state fMRI (rs-fMRI) and the regional homogeneity (ReHo) method to determine neural activity patterns in the two main clinical subgroups of PD (akinetic-rigid and tremor-dominant). Compared with healthy controls, akinetic-rigid (AR) subjects had increased ReHo mainly in right amygdala, left putamen, bilateral angular gyrus, bilateral medial prefrontal cortex (MPFC), and decreased ReHo in left post cingulate gyrus/precuneus (PCC/PCu) and bilateral thalamus. In contrast, tremor-dominant (TD) patients showed higher ReHo mostly in bilateral angular gyrus, left PCC, cerebellum_crus1, and cerebellum_6, while ReHo was decreased in right putamen, primary sensory cortex (S1), vermis_3, and cerebellum_4_5. These results indicate that AR and TD subgroups both represent altered spontaneous neural activity in default-mode regions and striatum, and AR subjects exhibit more changed neural activity in the mesolimbic cortex (amygdala) but TD in the cerebellar regions. Of note, direct comparison of the two subgroups revealed a distinct ReHo pattern primarily located in the striatal-thalamo-cortical (STC) and cerebello-thalamo-cortical (CTC) loops. Overall, our findings highlight the involvement of default mode network (DMN) and STC circuit both in AR and TD subtypes, but also underscore the importance of integrating mesolimbic-striatal and CTC loops in understanding neural systems of akinesia and rigidity, as well as resting tremor in PD. This study provides improved understanding of the pathophysiological models of different subtypes of PD. Copyright © 2014 Elsevier Ltd. All rights reserved.

Goiter and deaf mutism.

Science.gov (United States)

Thieme, E T

1975-08-01

The occurrence of deaf-mutism and goiter unassocaited with creatinism or mental retardation in euthyroid patients is known as Pendred's Syndrome. It is considered due to a single mutant recessive gene responsible for both the goiter and deafness. The penetrance is high, the intenseness of expressivity may vary within the same family and only one generation is affected. The extremely atypical hyperplasia seen in such goiters has been considered malignant. In 1956 the author reported a family in which 4 of 6 sibilings demonstrated Pendred's Syndrome. Three of the 4 had undergone thyroidectomy, two were considered to have carcinoma. Nineteen years later the family is again reported. The fourth sibling has recently undergone thyroidectomy. This thyroid demonstrated the same atypical hyperplasia as seen in the elder two siblings. The 19 year followup of this family has shown no evidence of recurrence or metastases, indicating that the atypical hyperplasia is probably not malignant. Pendred's Syndrome is described and certain suggestions are made for the counseling of the parents and the treatment and counseling of those children so afflicted.

The Teachers' Role in the Assessment of Selective Mutism and Anxiety Disorders

Science.gov (United States)

Martinez, Yvonne J.; Tannock, Rosemary; Manassis, Katharina; Garland, E. Jane; Clark, Sandra; McInnes, Alison

2015-01-01

Selective mutism (SM) is a childhood disorder characterized by failure to speak in social situations, despite there being an expectation to speak and the capacity to do so. There has been a focus on elucidating the differences between SM and anxiety disorder (ANX) in the recent literature. Although children with SM exhibit more symptoms at school…

Cerebellar Mutism Syndrome and Other Complications After Surgery in the Posterior Fossa in Adults

DEFF Research Database (Denmark)

Wibroe, Morten; Rochat, Per; Juhler, Marianne

2018-01-01

BACKGROUND: Cerebellar mutism syndrome (CMS) is rarely described in adults; however, data on self-assessed linguistic complications after posterior fossa surgery do not exist. METHODS: Through a prospective single-center study, data on 59 tumor operations in the posterior fossa were collected pre...

Can Adults Who Have Recovered from Selective Mutism in Childhood and Adolescence Tell Us Anything about the Nature of the Condition and/or Recovery from It?

Science.gov (United States)

Omdal, Heidi

2007-01-01

The literature on selective mutism provides little information on the child's own perspective. Six adults who had been selectively mute were interviewed about their childhood and adolescence. Data analysis led to identification of five themes, each of which has potentially important implications for teachers. (1) Origins of selective mutism: all…

The Current State of Empirical Support for the Pharmacological Treatment of Selective Mutism

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

2008-01-01

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,…

Treatment of Selective Mutism: Applications in the Clinic and School through Conjoint Consultation

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Mitchell, Angela D.; Kratochwill, Thomas R.

2013-01-01

The purpose of this study was to evaluate the effectiveness of a psychosocial approach to the treatment of Selective Mutism (SM). Four children with SM along with their parents and teachers participated in the study. A comprehensive assessment was completed; manualized treatment was implemented through a conjoint behavioral consultation approach,…

Behavioral Intervention to Treat Selective Mutism across Multiple Social Situations and Community Settings

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Lang, Russell; Regester, April; Mulloy, Austin; Rispoli, Mandy; Botout, Amanda

2011-01-01

We evaluated a behavioral intervention for a 9-year-old girl with selective mutism. The intervention consisted of role play and video self-modeling. The frequency of spoken initiations, responses to questions, and communication breakdowns was measured during three social situations (i.e., ordering in a restaurant, meeting new adults, and playing…

[Multilingualism and child psychiatry: on differential diagnoses of language disorder, specific learning disorder, and selective mutism].

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Tamiya, Satoshi

2014-01-01

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.

Ties of silence--Family lived experience of selective mutism in identical twins.

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Albrigtsen, Vårin; Eskeland, Benedicte; Mæhle, Magne

2016-04-01

This article is based on an in-depth interview with a pair of twins diagnosed with selective mutism and their parents 2 years after recovery. Selective mutism (SM) is a rare disorder, and identical twins sharing the condition are extremely rare. The twins developed SM simultaneously during their first year of school. The treatment and follow-up they received for several years are briefly described in this article. The interview explored the children's and their parents' narratives about the origin of the condition, the challenges it entailed in their daily lives, and what they found helpful in the treatment they were offered. In the interview, the children conveyed experiences that even the parents were unaware of and revealed examples of daily life-traumas for which they were unable to obtain support and help. The whole family was trapped in the silence. The twins and their parents emphasized different aspects in terms of what they believed were helpful. The implications of these findings for our understanding and treatment of children with SM are discussed, as well as the potential of service user involvement in child and adolescent mental health research. © The Author(s) 2015.

Association of grey matter changes with stability and flexibility of prediction in akinetic-rigid Parkinson's disease.

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Trempler, Ima; Binder, Ellen; El-Sourani, Nadiya; Schiffler, Patrick; Tenberge, Jan-Gerd; Schiffer, Anne-Marike; Fink, Gereon R; Schubotz, Ricarda I

2018-06-01

Parkinson's disease (PD), which is caused by degeneration of dopaminergic neurons in the midbrain, results in a heterogeneous clinical picture including cognitive decline. Since the phasic signal of dopamine neurons is proposed to guide learning by signifying mismatches between subjects' expectations and external events, we here investigated whether akinetic-rigid PD patients without mild cognitive impairment exhibit difficulties in dealing with either relevant (requiring flexibility) or irrelevant (requiring stability) prediction errors. Following our previous study on flexibility and stability in prediction (Trempler et al. J Cogn Neurosci 29(2):298-309, 2017), we then assessed whether deficits would correspond with specific structural alterations in dopaminergic regions as well as in inferior frontal cortex, medial prefrontal cortex, and the hippocampus. Twenty-one healthy controls and twenty-one akinetic-rigid PD patients on and off medication performed a task which required to serially predict upcoming items. Switches between predictable sequences had to be indicated via button press, whereas sequence omissions had to be ignored. Independent of the disease, midbrain volume was related to a general response bias to unexpected events, whereas right putamen volume correlated with the ability to discriminate between relevant and irrelevant prediction errors. However, patients compared with healthy participants showed deficits in stabilisation against irrelevant prediction errors, associated with thickness of right inferior frontal gyrus and left medial prefrontal cortex. Flexible updating due to relevant prediction errors was also affected in patients compared with controls and associated with right hippocampus volume. Dopaminergic medication influenced behavioural performance across, but not within the patients. Our exploratory study warrants further research on deficient prediction error processing and its structural correlates as a core of cognitive symptoms

Selective Mutism: A Team Approach to Assessment and Treatment in the School Setting

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Ponzurick, Joan M.

2012-01-01

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.…

Assessing Spoken Language Competence in Children with Selective Mutism: Using Parents as Test Presenters

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

2013-01-01

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…

The Importance of a Team Approach in Working Effectively with Selective Mutism: A Case Study

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Borger, Gary W.; Bartley, Dana L.; Armstrong, Norma; Kaatz, Debra; Benson, Dave

2007-01-01

Selective Mutism (SM) in children and adolescents is characterized by a persistent failure to speak in certain social situations including at school or with friends despite the ability to speak and comprehend language. Not due to a specific communication disorder, SM is actually a pervasive psychological problem that lies along the continuum of…

When silence is not golden: an integrated approach to selective mutism.

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McInnes, Alison; Manassis, Katharina

2005-08-01

Selective mutism (SM) is a rare disorder that is associated with both anxiety and communication impairments. Preliminary evidence suggests that educational attainment and development of social skills and self-esteem may be affected by SM in a significant proportion of cases. There is a critical need for cross-disciplinary research from the fields of speech-language pathology, psychiatry, and clinical psychology to develop protocols for assessing language and cognitive functioning in children with SM and developing interventions that address psychiatric, communicative, and social aspects of this disorder.

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

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

2017-01-01

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.

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

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JOSÉ ALBERTO GONÇALVES DA SILVA

1999-12-01

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.

The outcome of children with selective mutism following cognitive behavioral intervention: a follow-up study.

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Lang, Claudia; Nir, Ziv; Gothelf, Ayelet; Domachevsky, Shoshi; Ginton, Lee; Kushnir, Jonathan; Gothelf, Doron

2016-04-01

Selective mutism (SM) is a relatively rare childhood disorder and is underdiagnosed and undertreated. The purpose of the retrospective naturalistic study was to examine the long-term outcome of children with SM who were treated with specifically designed modular cognitive behavioral therapy (MCBT). Parents of 36 children who met diagnostic criteria of SM that received MCBT treatment were invited for a follow-up evaluation. Parents were interviewed using structured scales and completed questionnaires regarding the child, including the Selective Mutism Questionnaire (SMQ). Twenty-four subjects were identified and evaluated. Their mean age ± SD of onset of SM symptoms, beginning of treatment, and age at follow-up were 3.4 ± 1.4, 6.4 ± 3.1, and 9.3 ± 3.4 years, respectively. There was robust improvement from beginning of treatment to follow-up evaluation in SM, social anxiety disorder, and specific phobia symptoms. The recovery rate from SM was 84.2 %. SM-focused MCBT is feasible in children and possibly effective in inducing long-term reduction of SM and comorbid anxiety symptoms. • There are limited empirical data on selective mutism (SM) treatment outcome and specifically on cognitive-behavioral therapy, with the majority of studies being uncontrolled case reports of 1 to 2 cases each. • There is also limited data on the long-term outcome of children with SM following treatment. What is New: • Modular cognitive behavioral treatment is a feasible and possibly effective treatment for SM. Intervention at a younger age is more effective comparing to an older age. • Treatment for SM also decreases the rate of psychiatric comorbidities, including separation anxiety disorder and specific phobia.

An auditory-neuroscience perspective on the development of selective mutism

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Yael Henkin

2015-04-01

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.

Behavioral and Emotional Adjustment, Family Functioning, Academic Performance, and Social Relationships in Children with Selective Mutism

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

2004-01-01

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…

Evaluation of Children with Selective Mutism and Social Phobia: A Comparison of Psychological and Psychophysiological Arousal

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

2012-01-01

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),…

Playing with Gladys: A case study integrating drama therapy with behavioural interventions for the treatment of selective mutism.

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Oon, Phei Phei

2010-04-01

This case study examines an integrative approach combining drama therapy and the behavioural skill "shaping", as offered to Gladys, a 5-year-old girl diagnosed with selective mutism. This study found that shaping, when implemented in the context of play, with play as the primary reinforcer, elicited from Gladys vocalization and eventually speech within a very short time. Her vocalizations allowed her to enter dramatic play, which in turn propelled spontaneous speech. This article looks at how the three elements of dramatherapy - the playspace, role-playing and dramatic projection - brought about therapeutic changes for Gladys. Aside from spontaneous speech, Gladys also developed positive self-esteem and a heightened sense of spontaneity. Subsequently, these two qualities helped her generalize her speech to new settings on her own. Gladys's newly harnessed spontaneity further helped her become more sociable and resilient.This study advances the possibility of integrating a behavioural skill with drama therapy for the therapeutic benefits of a child with an anxiety-related condition like selective mutism.

Selective mutism and anxiety: a review of the current conceptualization of the disorder.

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Sharp, William G; Sherman, Colleen; Gross, Alan M

2007-01-01

Selective mutism (SM) is a rare and interesting condition that has been associated with a wide variety of childhood psychiatric conditions. Historically viewed as more of an oddity than a distinct diagnostic entity, early conceptualizations of the condition were based largely on case studies that tended to link SM with oppositional behavior. More recently, controlled studies have enhanced our understanding of SM. This review summarizes the current conceptualization of SM, highlighting evidence supporting the notion that SM is an anxiety-related condition.

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

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Henkin, Yael; Bar-Haim, Yair

2015-04-01

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. Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.

Selective mutism: more than social anxiety?

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Yeganeh, Robin; Beidel, Deborah C; Turner, Samuel M

2006-01-01

This study investigated the relationship between selective mutism (SM), social phobia (SP), oppositionality, and parenting styles. Twenty-one children with SP, 21 children with SM and SP, and 21 normal children ages 7-15, and the mother of each child, participated in an assessment of psychopathological factors potentially related to SM. Children with SM did not endorse higher levels of social anxiety than did children with SP, although clinicians gave higher severity ratings to those who had both disorders. In addition, although a dimensional measure of oppositionality (Eyberg Child Behavior Inventory) did not reveal group differences, there were significantly more diagnoses of oppositional defiant disorder among children with SM (29%) in comparison to children with SP alone (5%). With respect to parenting styles, there were no significant differences among parents of children with SM and the other groups, except that children with SP reported significantly less warmth/acceptance from parents than normal children. These data replicate previous findings that children with SM do not report greater social anxiety than other children with a SP diagnosis. Furthermore, they suggest that oppositional behaviors may be part of the clinical presentation of a subset of children with SM.

A Phenomenological Case Study of the Elementary to Secondary Transition for One Female Student Diagnosed with Selective Mutism

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Nashman-Smith, Mona

2017-01-01

Selective mutism (SM) is considered a communication and anxiety disorder that afflicts about 1% of students. The rarity of SM and the isolated cases of this condition has rendered the elementary to secondary school experience for a student with SM difficult to study. Utilizing a qualitative approach, this phenomenological case study examined the…

Practitioner Review: Psychosocial Interventions for Children with Selective Mutism--A Critical Evaluation of the Literature from 1990-2005

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Cohan, Sharon L.; Chavira, Denise A.; Stein, Murray B.

2006-01-01

Background: There have been several reports of successful psychosocial interventions for children with selective mutism (SM), a disorder in which a child consistently fails to speak in one or more social settings (e.g., school) despite speaking normally in other settings (e.g., home). The present literature review was undertaken in order to…

Comparing interventions for selective mutism: a pilot study.

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Manassis, Katharina; Tannock, Rosemary

2008-10-01

To examine the outcome within 6 to 8 months of medical and nonmedical intervention for children with severe selective mutism (SM). Children with SM (n = 17) and their mothers, seen in a previous study, attended follow-up appointments with a clinician. Obtained by maternal report were: treatment received, current diagnosis (based on semi-structured interview), speech in various environments, and global improvement. An independent clinician also rated global functioning. The diagnosis of SM persisted in 16 children, but significant symptomatic improvement was evident in the sample. All children had received school consultations. Children who had been treated with selective serotonin reuptake inhibitors (SSRI) (n = 10) showed greater global improvement, improvement in functioning, and improvement in speech outside the family than children who were unmedicated (n = 7). No differences were evident for children receiving and not receiving additional nonmedical intervention. The findings suggest the potential benefit of SSRI treatment in severe SM, but randomized comparative treatment studies are indicated.

Comorbidity and Family Factors Associated with Selective Mutism

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Brian A. Buzzella

2011-01-01

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.

Creutzfeldt-Jakob disease: A great masquerade in neurology, a rare case report from South India

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Sivaprakash Varadan

2015-01-01

Full Text Available Creutzfeldt-Jakob disease (CJD is a rare, fatal neurodegenerative disease caused by an infectious protein called prion and is characterized by spongiform changes, neuronal loss, reactive astrocytic proliferation, and accumulation of pathologic cellular protein. Clinical presentation of CJD is characterized by rapidly progressive dementia, neurologic symptoms and visual impairment, and the development of akinetic mutism, which can mimic many neurological conditions. The diagnosis is based on clinical presentation, electroencephalogram, and typical cerebrospinal fluid and magnetic resonance imaging (MRI findings. Literature on the incidence and prevalence of CJD is lacking in South India. We report the case of a 57-year-old woman with progressive dementia and typical neurologic symptoms, myoclonic jerks, and MRI findings of CJD. This case highlights the need for a high index of suspicion to diagnose CJD.

Successful combination immunotherapy of anti-gamma aminobutyric acid (GABA)A receptor antibody-positive encephalitis with extensive multifocal brain lesions.

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Fukami, Yuki; Okada, Hiroaki; Yoshida, Mari; Yamaguchi, Keiji

2017-08-31

A 78-year old woman who presented with akinetic mutism was admitted to our hospital. Brain MRI showed multifocal increased T 2 /FLAIR signal with extensive cortical-subcortical involvement. We suspected autoimmune encephalitis and the patient received methylprednisolone pulse. Her conscious level gradually recovered, but later relapsed again and presented with refractory status epilepticus. We treated her with intravenous immunoglobulin, plasma exchange and pulsed cyclophosphamide, with satisfactory response. A brain biopsy showed perivascular lymphocytic infiltrates and reactive gliosis. Anti-gamma aminobutyric acid (GABA) A receptor antibodies test came back to be positive after her recovery, and the diagnosis of anti-GABA A receptor antibody-positive encephalitis was made. This is a very rare case where brain biopsies were performed in a patient with anti-GABA A receptor antibody-positive encephalitis.

Bruxism Associated with Anoxic Encephalopathy: Successful Treatment with Baclofen

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A. Bruce Janati

2013-01-01

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.

Treatment of selective mutism: a 5-year follow-up study.

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Oerbeck, Beate; Overgaard, Kristin Romvig; Stein, Murray B; Pripp, Are Hugo; Kristensen, Hanne

2018-01-22

Selective mutism (SM) has been defined as an anxiety disorder in the diagnostic and statistical manual of mental disorders (DSM-5). Cognitive behavioral therapy (CBT) is the recommended approach for SM, but prospective long-term outcome studies are lacking. Reports from the children themselves, and the use of more global quality of life measures, are also missing in the literature. We have developed a school-based CBT intervention previously found to increase speech in a pilot efficacy study and a randomized controlled treatment study. Continued progress was found in our 1-year follow-up studies, where older age and more severe SM had a significant negative effect upon outcome. In the present study, we provide 5-year outcome data for 30 of these 32 children with SM who completed the same CBT for mean 21 weeks (sd 5, range 8-24) at mean age 6 years (10 boys). Mean age at the 5-year follow-up was 11 years (range 8-14). Outcome measures were diagnostic status, the teacher- and parent-rated selective mutism questionnaires, and child rated quality of life and speaking behavior. At the 5-year follow-up, 21 children were in full remission, five were in partial remission and four fulfilled diagnostic criteria for SM. Seven children (23%) fulfilled criteria for social phobia, and separation anxiety disorder, specific phobia and/or enuresis nocturna were found in a total of five children (17%). Older age and severity at baseline and familial SM were significant negative predictors of outcome. Treatment gains were maintained on the teacher- and parent questionnaires. The children rated their overall quality of life as good. Although most of them talked outside of home, 50% still experienced it as somewhat challenging. These results point to the long-term effectiveness of CBT for SM, but also highlight the need to develop more effective interventions for the subset of children with persistent symptoms.Clinical trials registration NCT01002196.

Joint Attention in Parent-Child Dyads Involving Children with Selective Mutism: A Comparison between Anxious and Typically Developing Children

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Nowakowski, Matilda E.; Tasker, Susan L.; Cunningham, Charles E.; McHolm, Angela E.; Edison, Shannon; St. Pierre, Jeff; Boyle, Michael H.; Schmidt, Louis A.

2011-01-01

Although joint attention processes are known to play an important role in adaptive social behavior in typical development, we know little about these processes in clinical child populations. We compared early school age children with selective mutism (SM; n = 19) versus mixed anxiety (MA; n = 18) and community controls (CC; n = 26) on joint…

Attention in selective mutism--an exploratory case-control study.

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Oerbeck, Beate; Kristensen, Hanne

2008-01-01

The aim of the study was to explore the association between selective mutism (SM) and attention. In SM social anxiety seems central but language impairment and motor problems are also reported. Attention problems have been described in parental behavioral ratings, while neuropsychological studies are lacking. A neuropsychological test (the Trail Making Test) and parental ratings of attention- and anxiety problems were administered to a clinical sample of 23 children with SM (aged 7-16 years, 12 boys and 11 girls) and 46 non-referred matched controls. The SM group differed from controls on the Trail Making Test, but the group difference disappeared, when controlling for motor function and IQ. Parental ratings of attention problems were not significantly associated with the neuropsychological attention measure. Neuropsychological studies of attention controlled for IQ and motor function are needed as well as tests that measure different aspects of attention.

An Examination of Fluoxetine for the Treatment of Selective Mutism Using a Nonconcurrent Multiple-Baseline Single-Case Design Across 5 Cases.

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Barterian, Justin A; Sanchez, Joel M; Magen, Jed; Siroky, Allison K; Mash, Brittany L; Carlson, John S

2018-01-01

This study examined the utility of fluoxetine in the treatment of 5 children, aged 5 to 14 years, diagnosed with selective mutism who also demonstrated symptoms of social anxiety. A nonconcurrent, randomized, multiple-baseline, single-case design with a single-blind placebo-controlled procedure was used. Parents and the study psychiatrist completed multiple methods of assessment including Direct Behavior Ratings and questionnaires. Treatment outcomes were evaluated by calculating effect sizes for each participant as an individual and for the participants as a group. Information regarding adverse effects with an emphasis on behavioral disinhibition and ratings of parental acceptance of the intervention was gathered. All 5 children experienced improvement in social anxiety, responsive speech, and spontaneous speech with medium to large effect sizes; however, children still met criteria for selective mutism at the end of the study. Adverse events were minimal, with only 2 children experiencing brief occurrences of minor behavioral disinhibition. Parents found the treatment highly acceptable.

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

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Nikolay Natchev

2016-09-01

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.

The First Report of a Patient with Probable Variant Creutzfeldt-Jakob Disease in Turkey

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Demet Özbabalık Adapınar

2011-12-01

Full Text Available Variant Creutzfeldt-Jakob disease (vCJD was first reported in the UK in 1996. Here, we report the first Turkish case of vCJD. A 47-year-old man, who has never lived outside of Turkey and had had no transfusion, was admitted to the University Hospital with speech disorder, cognitive decline and ataxia following depression, irritability, and personality change. The immunoassay of the 14-3-3 protein in the cerebrospinal fluid was negative. Brain magnetic resonance imaging revealed high-signal lesions involving the bilateral caudate and lentiform nucleus on T2- and diffusion-weighted imaging. The patient developed akinetic mutism 10 months after disease onset. The clinical presentation and neuroimaging findings were compatible with the vCJD cases reported since 1996 and met the World Health Organization’s case definition for probable vCJD.

Behavioral and Socio-Emotional Functioning in Children with Selective Mutism: A Comparison with Anxious and Typically Developing Children across Multiple Informants

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Carbone, Diana; Schmidt, Louis A.; Cunningham, Charles C.; McHolm, Angela E.; Edison, Shannon; St. Pierre, Jeff; Boyle, Michael H.

2010-01-01

We examined differences among 158 children, 44 with selective mutism (SM; M = 8.2 years, SD = 3.4 years), 65 with mixed anxiety (MA; M = 8.9 years, SD = 3.2 years), and 49 community controls (M = 7.7 years, SD = 2.6 years) on primary caregiver, teacher, and child reports of behavioral and socio-emotional functioning. Children with SM were rated…

Reduced auditory processing capacity during vocalization in children with Selective Mutism.

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Arie, Miri; Henkin, Yael; Lamy, Dominique; Tetin-Schneider, Simona; Apter, Alan; Sadeh, Avi; Bar-Haim, Yair

2007-02-01

Because abnormal Auditory Efferent Activity (AEA) is associated with auditory distortions during vocalization, we tested whether auditory processing is impaired during vocalization in children with Selective Mutism (SM). Participants were children with SM and abnormal AEA, children with SM and normal AEA, and normally speaking controls, who had to detect aurally presented target words embedded within word lists under two conditions: silence (single task), and while vocalizing (dual task). To ascertain specificity of auditory-vocal deficit, effects of concurrent vocalizing were also examined during a visual task. Children with SM and abnormal AEA showed impaired auditory processing during vocalization relative to children with SM and normal AEA, and relative to control children. This impairment is specific to the auditory modality and does not reflect difficulties in dual task per se. The data extends previous findings suggesting that deficient auditory processing is involved in speech selectivity in SM.

Neurologic Complications of Transplantation.

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Dhar, Rajat

2018-02-01

Neurologic disturbances including encephalopathy, seizures, and focal deficits complicate the course 10-30% of patients undergoing organ or stem cell transplantation. While much or this morbidity is multifactorial and often associated with extra-cerebral dysfunction (e.g., graft dysfunction, metabolic derangements), immunosuppressive drugs also contribute significantly. This can either be through direct toxicity (e.g., posterior reversible encephalopathy syndrome from calcineurin inhibitors such as tacrolimus in the acute postoperative period) or by facilitating opportunistic infections in the months after transplantation. Other neurologic syndromes such as akinetic mutism and osmotic demyelination may also occur. While much of this neurologic dysfunction may be reversible if related to metabolic factors or drug toxicity (and the etiology is recognized and reversed), cases of multifocal cerebral infarction, hemorrhage, or infection may have poor outcomes. As transplant patients survive longer, delayed infections (such as progressive multifocal leukoencephalopathy) and post-transplant malignancies are increasingly reported.

A Vietnamese man with selective mutism: the relevance of multiple interacting 'cultures' in clinical psychiatry.

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Hollifield, Michael; Geppert, Cynthia; Johnson, Yuam; Fryer, Carol

2003-09-01

Multiple cultural variables have effects on the psychobiology and behavioral manifestations of illness, as do patient and physician perceptions of illness. The interaction among these variables is at the heart of clinical psychiatry. This case of a Vietnamese man with selective mutism underscores the relevance of the 'cultures' of medicine, psychiatry, and war and trauma on the manifestations of illness and illness perceptions by patient and physician. The discussion focuses on how these cultures interact and play a crucial role in formulating diagnosis and treatment planning. Suggestions are given for shifts in medical education that will encourage relevant cultural paradigms to make their way into educational and clinical systems, which in turn should improve cultural competence in clinical psychiatry.

[Mutism and acute behavioral disorders revealing MELAS syndrome].

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Coomans, H; Barroso, B; Bertandeau, E; Bonnan, M; Dakar, A; Demasles, S; Garraud, S; Krim, E; Martin-Négrier, M-L

2011-11-01

MELAS syndrome (mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes) is a rare genetic mitochondrial disease which can cause cerebral (cerebrovascular accident, migraine, mental deterioration..), sensorial (bilateral symmetrical deafness) and peripheral (muscular involvement, neuropathy) disorders potentially associated with diabetes, renal or cardiac disorders, or growth retardation. Eighty percent of the patients have the 3243 A>G mutation in the leucine RNA transfer gene. Clinical manifestations leading to discovery of the mutation can be extremely varied, affecting patients of different age groups. We report the case of a 49-year-old man who presented acute fits of confusion followed by mutism and praxic disorders. History taking revealed recently diagnosed type 2 diabetes, axonal neuropathy, and bilateral symmetrical deafness requiring hearing aids. The initial MRI showed FLAIR sequences with bi-parietal abnormalities, no signs of recent stroke on the DW/B10000 sequences, and basal ganglia calcifications. Blood tests and morphological findings ruled out a vascular origin. Search for lactic acidosis remained constantly negative in blood samples despite positive cerebrospinal fluid samples (N×3). The 3243 A>G mitochondrial DNA mutation was identified. The neuropsychological evaluation revealed a serious dysexecutive syndrome with a major impact on the patient's self sufficiency. Neurocognitive disorders are not common in MELAS syndrome. Brain MRI results and the presence of extra-neurological signs can be helpful for diagnosis. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

Tc-99m-bicisate (ECD)-brain-SPECT in rapidly progressive dementia

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Marienhagen, J.; Eilles, C.; Weingaertner, U.; Blaha, L.; Zerr, I.; Poser, S.

1999-01-01

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.) [de

Selective mutism. A school-based cross-sectional study from Turkey.

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Karakaya, I; Sişmanlar, S G; Oç, O Y; Memik, N C; Coşkun, A; Ağaoğlu, B; Yavuz, C I

2008-03-01

The aim of this study is to examine the prevalence of selective mutism (SM) in Kocaeli, Turkey. Kindergarten, first, second and third grade students of all public/private schools within the city were included in the study. "SM screening forms" prepared on basis of DSM-IV were submitted to classroom teachers in all these schools asking whether they had any students meeting such symptoms. About 84.51% of the schools returned forms covering 64,103 children. Five hundred and twenty six of these children were thought to have symptoms of SM by their teachers. After their DSM-IV based clinical evaluation by a child and adolescent psychiatrist, only 21 children were diagnosed as SM. Among the SM group, three were in the kindergarten, 15 were in the first grade and three were in the second grade. Twelve of the children were male and nine were female (male: female ratio is 1.3:1). In this cross-sectional study, 0.83% of children were reported to have SM symptoms by their teachers. After the clinical evaluation of these children, the prevalence rate of SM was found to be 0.033%.

Selective mutism: a review and integration of the last 15 years.

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Viana, Andres G; Beidel, Deborah C; Rabian, Brian

2009-02-01

Selective mutism (SM) is a rare childhood disorder characterized by a lack of speech in one or more settings in which speaking is socially expected. A comprehensive and uniform theory about the etiology, assessment, and treatment of SM does not exist. Historically, varying definitions and criteria have been applied to children with SM, therefore making comparisons between studies somewhat difficult. Accumulating findings on the phenomenology of SM point to a complex and multidetermined etiology. Developmental psychopathology represents a useful heuristic for conceptualization of SM and serves as an integrative framework for organizing the sometimes disparate findings that permeate the SM literature. The purpose of this review is to summarize the literature on SM, including phenomenology, assessment, and treatment, with the main goals of clarifying its clinical presentation, offering a theoretical understanding of SM from a developmental psychopathology perspective, and highlighting both research and practice gaps that may exist. Recommendations for future research are made with the goal of expanding the current knowledge base on the etiology of SM.

Selective Mutism: The Fraternal Twin of Childhood Social Phobia.

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Gensthaler, Angelika; Maichrowitz, Verena; Kaess, Michael; Ligges, Marc; Freitag, Christine M; Schwenck, Christina

Selective mutism (SM) is an anxiety disorder with a close link to childhood social phobia (SP). Our studies compare behavioral problem profiles in children and adolescents with SM and SP and control groups and assess the comorbidity patterns of SM and SP. Participants aged 3-18 years with SM (n = 95), SP (n = 74) and internalizing disorders (INT, n = 46) and a typically developing control group (CG, n = 119) were assessed with the Child Behavior Checklist (CBCL); adolescents were additionally assessed with the Youth Self-Report (YSR). Comorbidity was assessed in SM and SP participants with a diagnostic interview. SP was detected in 94% of children with SM. SM participants showed different behavioral and psychiatric symptoms than SP: they were more frequently affected by lifetime separation anxiety disorder (SM: 45%, SP: 26%) and oppositional defiant disorder (SM: 22%, SP: 5%), and less by generalized anxiety disorder (SM: 6%, SP: 20%) and major depression (SM: 12%, SP: 26%). Adolescents with SM showed high rates of agoraphobia (SM 27%; SP 10%) and more social problems (YSR), and were more withdrawn (CBCL, YSR) than those with SP alone. Specific behavioral problems of SM and SP compared to INT and CG were observed. SM and SP represent separate but closely related disorders, distinct from other INT and CG, with specific patterns of lifetime comorbidities. © 2016 S. Karger AG, Basel.

Selective mutism: follow-up study 1 year after end of treatment.

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Oerbeck, Beate; Stein, Murray B; Pripp, Are H; Kristensen, Hanne

2015-07-01

Cognitive behavioral therapy (CBT) is generally considered the recommended approach for selective mutism (SM). Prospective follow-up studies of treated SM and predictors of outcome are scarce. We have developed a CBT home and school-based intervention for children with SM previously found to increase speech in a pilot efficacy study and in a randomized controlled treatment study. In the present report we provide outcome data 1 year after having completed the 6-month course of CBT for 24 children with SM, aged 3-9 years (mean age 6.5 years, 16 girls). Primary outcome measures were the teacher rated School Speech Questionnaire (SSQ) and diagnostic status. At follow-up, no significant decline was found on the SSQ scores. Age and severity of SM had a significant effect upon outcome, as measured by the SSQ. Eight children still fulfilled diagnostic criteria for SM, four were in remission, and 12 children were without diagnosis. Younger children improved more, as 78% of the children aged 3-5 years did not have SM, compared with 33% of children aged 6-9 years. Treatment gain was upheld at follow-up. Greater improvement in the younger children highlights the importance of an early intervention.

Ericksonian hypnotherapy for selective mutism: A single-case study.

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Cavarra, Mauro; Brizio, Adelina; Gava, Nicoletta

2017-01-16

Children affected by selective mutism don't speak in contexts that are unfamiliar to them or in which speaking is expected or required (e.g. school, kindergarten…). Such disorder interferes with the child's normal activities, may have invalidating consequences in the long run if left untreated, is associated to anxious conditions and is considered hard to treat. Contemporary research is still in need of methodologically rigorous outcome studies and the results described in the small number of published randomized controlled trials and retrospective studies indicate cognitive-behavioral interventions lasting 20-24 sessions as the best therapeutic option. This case study, involving a 7-year-old girl, aims at providing preliminary evidence on the effectiveness of Ericksonian hypnosis in the treatment of this condition. A brief review of current evidence is provided. The case was treated by a licensed hypnotherapist, specialized in family therapy, in 5 sessions during the course of 3 months. After 3 months the symptoms of the client were resolved and the diagnosis was no longer applicable. Other improvements regarded her mood, social skills and school performance.  Conclusions: Ericksonian Hypnotherapy lead to the remission of the disorder and to the improvement of the general well being of the client in 5 sessions, a much briefer time span compared to what is reported in current literature. This paper represents the first step in the elaboration of replicable and reliable intervention principles.

Parental adjustment, parenting attitudes and emotional and behavioral problems in children with selective mutism.

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Alyanak, Behiye; Kılınçaslan, Ayşe; Harmancı, Halime Sözen; Demirkaya, Sevcan Karakoç; Yurtbay, Tülin; Vehid, Hayriye Ertem

2013-01-01

The present study investigated emotional and behavioral problems in children with selective mutism (SM) along with the psychological adjustment and parenting attitudes of their mothers and fathers. Participants included 26 children with SM (mean age = 8.11 ± 2.11 years), 32 healthy controls (mean age = 8.18 ± 2.55 years) and the parents of all children. Children with SM displayed higher problem scores than controls in a variety of emotional and behavioral parameters. They predominantly displayed internalizing problems, whereas aggressive and delinquent behavior was described among a subsample of the children. Significant differences existed between the SM and control groups only in paternal psychopathology, which included anxiety and depression. They did not differ with respect to maternal psychological distress or mother or father reported parental attitudes. Another important result of the present study was that the severity of emotional and behavioral problems of children with SM was correlated with maternal psychopathology but not paternal psychopathology. Copyright © 2012 Elsevier Ltd. All rights reserved.

Identifying Treatment Response of Sertraline in a Teenager with Selective Mutism using Electrophysiological Neuroimaging.

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Eugene, Andy R; Masiak, Jolanta

2016-06-01

Selective Mutism is described as the inability to verbally express oneself in anxiety provoking social situations and may result in awkward social interactions in school-aged children. In this case-report we present the baseline electrophysiological neuroimaging results and after treatment with Sertraline for 6-weeks. A 20-channel EEG event-related potential recording was acquired during an internal voice task at baseline prior to the initiation of 50mg of Sertraline and then repeated 6-weeks after treatment with Sertraline. EEG signals were processed for movement, eye-blink, and muscle artifacts and ERP signal averaging was completed. ERPs were analyzed using Standard Low Resolution Brain Electromagnetic Tomography (sLORETA). At baseline, Sertraline increased the neuronal activation in the middle temporal gyrus and the anterior cingulate gyrus from baseline in the patient following 6-weeks of treatment. Our findings suggest that electrophysiological neuroimaging may provide a creative approach for personalizing medicine by providing insight to the pharmacodynamics of antidepressants.

Bilateral anterior thalamic low densities in descending transtentorial herniation

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Nagashima, Chikao; Watanabe, Takao

1985-02-01

Round, well-demarcated, symmetrical low densities in a bilateral thalamus in a case of descending transtentorial herniation due secondarily to acute traumatic left subdural hematoma are reported. An 8-year-old boy, on whom emergency surgery was refused by his parents, showed a marked shift due to the hematoma on admission; this was followed by a low density in the left PCA territory and round, equivocal hypodensities in the anterior thalamus 44 hours post-trauma. The equivocal hypodensities became definite, well-demarcated, round low densities situated symmetrically in the anterior thalamus on the 39th day post-trauma. Akinetic mutism was noted at this time. The symmetrical low densities and the PCA-territory low density persisted as late as the 39th day post-trauma, suggesting infarcts. The downward stretch of the bilateral thalamoperforators, which was effected by a narrowing of the interpeduncular fossa with an approximation of the bilateral perforators, plus a downward shift of the PCA due to descending transtentorial herniation, was assumed to be the mechanism involved. (author).

Mutism and auditory agnosia due to bilateral insular damage--role of the insula in human communication.

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

1995-03-01

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.

Treatment of selective mutism based on cognitive behavioural therapy, psychopharmacology and combination therapy - a systematic review.

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Østergaard, Kasper Rud

2018-02-15

Selective mutism (SM) is a debilitating childhood anxiety disorder characterized by a persistent lack of speech in certain social settings and is considered hard to treat. Cognitive behavioral therapy (CBT) and pharmacological treatments are the best described treatments in the literature. To test whether there is evidence on treatment based on CBT, medication or a combination of these. Systematic and critical review of the literature on CBT and/or pharmacological treatments of SM. Literature was sought on PubMed, Embase and Psycinfo in March 2017. Of the included studies, six examined CBT, seven pharmacologic treatment and two a combination of these. Using CBT 53/60 children improved symptomatically whilst respectively 55/67 and 6/7 improved using pharmacologic- and combination-treatment. Pharmacologic treatment and especially CBT showed promising results supported by some degree of evidence, which combination treatment lacks. Yet small numbers, few RCTs, heterogeneous study designs, lack of consistent measures, short treatment and follow-up periods, generally limits the evidence. This needs focus in future research.

The use of medication in selective mutism: a systematic review.

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Manassis, Katharina; Oerbeck, Beate; Overgaard, Kristin Romvig

2016-06-01

Despite limited evidence, selective serotonin reuptake inhibitors (SSRIs) and monoamine oxidase inhibitors (MAOIs) are used to reduce symptoms of selective mutism (SM) in children unresponsive to psychosocial interventions. We review existing evidence for the efficacy of these medications, limitations of the literature, and resulting treatment considerations. Bibliographic searches were conducted in Medline, Embase, PsycInfo, Web of Science and Cochrane up to June 2015. Two reviewers independently sought studies of children with SM as primary psychiatric diagnosis, which reported response to medication treatment. Abstracts were limited to those reporting original data. Two reviewers independently assessed the ten papers reporting on >2 subjects regarding study design, key results, and limitations. Heterogeneity of designs mandated a descriptive summary. Symptomatic improvement was found for 66/79 children treated with SSRIs and 4/4 children treated with phenelzine. Only 3/10 studies had unmedicated comparison groups and only two were double-blinded. This review may be affected by publication bias, missed studies, and variability of outcome measures in included studies. Although there is some evidence for symptomatic improvement in SM with medication, especially SSRIs, it is limited by small numbers, lack of comparative trials, lack of consistent measures, and lack of consistent reporting on tolerability. The clinician must weigh this paucity of evidence against the highly debilitating nature of SM, and its adverse effects on the development of those children whose progress with psychosocial interventions is limited or very slow. Studies of optimal dosage and timing of medications in relation to psychosocial treatments are also needed.

Selective mutism: are primary care physicians missing the silence?

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Schwartz, Richard H; Freedy, Alicia S; Sheridan, Michael J

2006-01-01

To survey parents of children with selective mutism (SM) in regard to (1) the role of the primary care physician in the diagnosis of SM; (2) the social and school consequences of SM; and (3) their opinion of the effectiveness of different treatment modalities, a 39-item written survey was mailed to 27 parents with at least one child diagnosed with SM on the basis of diagnostic and statistical manual IV-text revision (DSM IV-TR) criteria. Twenty-seven parents (100%), with a total of 33 children with SM, completed the survey. There were 24 girls and 9 boys. The mean age when parents had strong concerns about symptoms of SM was 3.8 years, but diagnosis did not occur until nearly a year later. Twenty-three (69.7%) of the children with SM were never diagnosed accurately or referred by their primary care physicians. SM caused important school/social problems for 17 (51.5%) of the children. Speech therapy was provided for 36.4% of children and was thought to have been helpful for 30% of them. Behavior modification was the treatment for 45.5% of children and perceived to be helpful for 66.7% of them. Selective serotonin re-uptake inhibitor pharmacotherapy was prescribed for 17 (51.5%) of the children and believed to be effective for 11 (65%) of them. Primary care physicians in this study rarely diagnosed accurately or referred children with SM in a timely fashion, even though symptoms of the condition were generally very apparent and parents had expressed concern. Behavioral modification, pharmacotherapy with SSRIs, and early intervention are viable treatment options. Early diagnosis is key to preventing long-term effects of this condition.

Prevalence and description of selective mutism in immigrant and native families: a controlled study.

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Elizur, Yoel; Perednik, Ruth

2003-12-01

To assess the incidence of selective mutism (SM) in West Jerusalem's state preschools and evaluate social anxiety/phobia disposition (SAP), social competence (SC), markers of neurodevelopmental delay/disorder (NDD), mothers' psychological adjustment, and marital conflict in immigrant and native children with SM and their matched controls. Mothers of 9 immigrant and 10 native children with SM and their matched controls completed questionnaires evaluating themselves, their marriages, and their children. A response rate of 30% (19/64) was obtained. The general prevalence of SM was 0.76%, while the rate among immigrants was 2.2%. Except for mothers' adjustment, all immigrant/native group effects were significant. There were significant interactions between the SM/control and immigrant/native groups for SAP, NDD, and SC. Immigrant children with SM had higher SAP and SC scores and lower NDD scores than native children with SM. This study distinguished between homogenous (socially anxious) and comorbid children with SM. In this sample, the disorder appeared to be associated with a combination of a specific diathesis (SAP) with intrinsic (NDD) and/or environmental (family immigration) vulnerabilities. Marital discord appeared to be a general risk factor for SM.

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

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Muris, Peter; Ollendick, Thomas H

2015-06-01

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.

Compact akinetic swept source optical coherence tomography angiography at 1060 nm supporting a wide field of view and adaptive optics imaging modes of the posterior eye.

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Salas, Matthias; Augustin, Marco; Felberer, Franz; Wartak, Andreas; Laslandes, Marie; Ginner, Laurin; Niederleithner, Michael; Ensher, Jason; Minneman, Michael P; Leitgeb, Rainer A; Drexler, Wolfgang; Levecq, Xavier; Schmidt-Erfurth, Ursula; Pircher, Michael

2018-04-01

Imaging of the human retina with high resolution is an essential step towards improved diagnosis and treatment control. In this paper, we introduce a compact, clinically user-friendly instrument based on swept source optical coherence tomography (SS-OCT). A key feature of the system is the realization of two different operation modes. The first operation mode is similar to conventional OCT imaging and provides large field of view (FoV) images (up to 45° × 30°) of the human retina and choroid with standard resolution. The second operation mode enables it to optically zoom into regions of interest with high transverse resolution using adaptive optics (AO). The FoV of this second operation mode (AO-OCT mode) is 3.0° × 2.8° and enables the visualization of individual retinal cells such as cone photoreceptors or choriocapillaris. The OCT engine is based on an akinetic swept source at 1060 nm and provides an A-scan rate of 200 kHz. Structural as well as angiographic information can be retrieved from the retina and choroid in both operational modes. The capabilities of the prototype are demonstrated in healthy and diseased eyes.

Abnormalities in auditory efferent activities in children with selective mutism.

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Muchnik, Chava; Ari-Even Roth, Daphne; Hildesheimer, Minka; Arie, Miri; Bar-Haim, Yair; Henkin, Yael

2013-01-01

Two efferent feedback pathways to the auditory periphery may play a role in monitoring self-vocalization: the middle-ear acoustic reflex (MEAR) and the medial olivocochlear bundle (MOCB) reflex. Since most studies regarding the role of auditory efferent activity during self-vocalization were conducted in animals, human data are scarce. The working premise of the current study was that selective mutism (SM), a rare psychiatric disorder characterized by consistent failure to speak in specific social situations despite the ability to speak normally in other situations, may serve as a human model for studying the potential involvement of auditory efferent activity during self-vocalization. For this purpose, auditory efferent function was assessed in a group of 31 children with SM and compared to that of a group of 31 normally developing control children (mean age 8.9 and 8.8 years, respectively). All children exhibited normal hearing thresholds and type A tympanograms. MEAR and MOCB functions were evaluated by means of acoustic reflex thresholds and decay functions and the suppression of transient-evoked otoacoustic emissions, respectively. Auditory afferent function was tested by means of auditory brainstem responses (ABR). Results indicated a significantly higher proportion of children with abnormal MEAR and MOCB function in the SM group (58.6 and 38%, respectively) compared to controls (9.7 and 8%, respectively). The prevalence of abnormal MEAR and/or MOCB function was significantly higher in the SM group (71%) compared to controls (16%). Intact afferent function manifested in normal absolute and interpeak latencies of ABR components in all children. The finding of aberrant efferent auditory function in a large proportion of children with SM provides further support for the notion that MEAR and MOCB may play a significant role in the process of self-vocalization. © 2013 S. Karger AG, Basel.

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

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Anyfantakis Dimitrios

2009-11-01

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.

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

Science.gov (United States)

2009-01-01

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. PMID:19946578

Risk factors for development of postoperative cerebellar mutism syndrome in children after medulloblastoma surgery.

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Pols, San Y C V; van Veelen, Marie Lise C; Aarsen, Femke K; Gonzalez Candel, Antonia; Catsman-Berrevoets, Coriene E

2017-07-01

OBJECTIVE Postoperative cerebellar mutism syndrome (pCMS) occurs in 7%-50% of children after cerebellar tumor surgery. Typical features include a latent onset of 1-2 days after surgery, transient mutism, emotional lability, and a wide variety of motor and neurobehavioral abnormalities. Sequelae of this syndrome usually persist long term. The principal causal factor is bilateral surgical damage (regardless of tumor location) to any component of the proximal efferent cerebellar pathway, which leads to temporary dysfunction of cerebral cortical regions as a result of diaschisis. Tumor type, cerebellar midline location, and brainstem involvement are risk factors for pCMS that have been identified repeatedly, but they do not explain its latent onset. Ambiguous or negative results for other factors, such as hydrocephalus, postoperative meningitis, length of vermian incision, and tumor size, have been reached. The aim of this study was to identify perioperative clinical, radiological, and laboratory factors that also increase risk for the development of pCMS. The focus was on factors that might explain the delayed onset of pCMS and thus might provide a time window for taking precautionary measures to prevent pCMS or reduce its severity. The study was focused specifically on children who had undergone surgery for medulloblastoma. METHODS In this single-center retrospective cohort study, the authors included 71 children with medulloblastoma, 28 of whom developed pCMS after primary resection. Clinical and laboratory data were collected prospectively and analyzed systematically. Variables were included for univariate and multivariate analysis. RESULTS Univariate regression analysis revealed 7 variables that had a significant influence on pCMS onset, namely, tumor size, maximum tumor diameter > 5 cm, tumor infiltration or compression of the brainstem, significantly larger decreases in hemoglobin (p = 0.010) and hematocrit (p = 0.003) in the pCMS group after surgery than in the

Nitrogen limitation and slow drying induce desiccation tolerance in conjugating green algae (Zygnematophyceae, Streptophyta from polar habitats.

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Martina Pichrtová

Full Text Available Filamentous Zygnematophyceae are typical components of algal mats in the polar hydro-terrestrial environment. Under field conditions, they form senescent vegetative cells, designated as pre-akinetes, which are tolerant to desiccation and osmotic stress.Pre-akinete formation and desiccation tolerance was investigated experimentally under monitored laboratory conditions in four strains of Arctic and Antarctic isolates with vegetative Zygnema sp. morphology. Phylogenetic analyses of rbcL sequences revealed one Arctic strain as genus Zygnemopsis, phylogenetically distant from the closely related Zygnema strains. Algae were cultivated in liquid or on solidified medium (9 weeks, supplemented with or lacking nitrogen. Nitrogen-free cultures (liquid as well as solidified consisted of well-developed pre-akinetes after this period. Desiccation experiments were performed at three different drying rates (rapid: 10% relative humidity, slow: 86% rh and very slow; viability, effective quantum yield of PS II, visual and ultrastructural changes were monitored. Recovery and viability of pre-akinetes were clearly dependent on the drying rate: slower desiccation led to higher levels of survival. Pre-akinetes survived rapid drying after acclimation by very slow desiccation.The formation of pre-akinetes in polar Zygnema spp. and Zygnemopsis sp. is induced by nitrogen limitation. Pre-akinetes, modified vegetative cells, rather than specialized stages of the life cycle, can be hardened by mild desiccation stress to survive rapid drying. Naturally hardened pre-akinetes play a key role in stress tolerance and dispersal under the extreme conditions of polar regions, where sexual reproduction and production of dormant stages is largely suppressed.

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

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Michelle Molina

2012-12-01

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.

Selective mutism: a home-and kindergarten-based intervention for children 3-5 years: a pilot study.

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

2012-07-01

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.

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

Science.gov (United States)

Muris, Peter; Hendriks, Eline; Bot, Suili

2016-02-01

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.

Selective mutism: a consensus based care pathway of good practice.

Science.gov (United States)

Keen, D V; Fonseca, S; Wintgens, A

2008-10-01

Selective mutism (SM) now acknowledged as an anxiety condition, tends to be a poorly understood, highly complex and vastly under-recognised clinical entity. Children with SM are a vulnerable group as the condition is not the remit of any one professional group. This inevitably leads to delay in formal diagnosis and management. There is a lack of systematic research on which to base guidelines for management. To develop, agree and validate key principles underlying the management of SM through a consensus process involving international experts, in order to create a local care pathway. A local multi-agency consultation process developed 11 statements, which were felt to be the key principles underpinning a potential care pathway for managing SM. Thirteen recognised experts from North America, Europe and Australia participated in a modified Delphi process involving two rounds using a Likert-scale and free commentary. Both quantitative and qualitative analyses were used in the validation or revision of the statements at each stage. Response rates were 100% for Round 1 and 84.6% for Round 2. Despite the differing professional backgrounds and service contexts, by successive revision and/or revalidation of statements, it was possible to arrive at a consensus about key principles relating to early recognition, assessment and intervention. The agreed key principles are presented together with the resulting local care pathway. Through a Delphi process, agreement was reached by a multidisciplinary group of professionals, on key principles that underpin the timely identification, assessment and management of children with SM. These include the potential for staff in school/preschool settings to identify SM and that intervention programmes should generally be based in these settings. Children with SM should receive assessment for possible coexisting disorders, whether developmental, emotional or behavioural and additional specific intervention given for these. Agreement was

Children's and parent's psychological profiles in selective mutism and generalized anxiety disorder: a clinical study.

Science.gov (United States)

Capozzi, Flavia; Manti, Filippo; Di Trani, Michela; Romani, Maria; Vigliante, Miriam; Sogos, Carla

2017-10-28

Selective mutism (SM) is classified in DSM-5 as an anxiety disorder. The aim of the study was to investigate the psychological features of children with SM and their parental psychological profiles, compared to generalized anxiety disorder (GAD) children and their parents. The parents of 26 preschool children with SM and 32 with children with GAD filled out the child behavior check list for 1½-5 years (CBCL1½-5) and the symptom checklist-90-revised (SCL-90-R). Information about the children and their parents' histories was collected through clinical interviews. Children with SM scored higher than children with GAD on the CBCL1½-5 withdrawn scale and lower on the attention problems, aggressive behavior, and externalizing problems scales. Mothers of children with SM scored higher on the SCL-90-R obsessive-compulsive subscale and Global Severity Index than mothers of children with GAD, while fathers of children with SM scored higher on the SCL-90-R Phobic Anxiety subscale and on the Global Severity Index than fathers of children with GAD. Parents of children with SM displayed a greater presence of stressful life events than parents of children with GAD. Data appeared to confirm that SM and GAD share a common anxious core, though some differences in the children's psychological profiles and the parents' history and personality emerged. Future research should focus on the role of external factors, such as parent-child relationship, in the development of SM.

Practitioner review: Psychosocial interventions for children with selective mutism: a critical evaluation of the literature from 1990-2005.

Science.gov (United States)

Cohan, Sharon L; Chavira, Denise A; Stein, Murray B

2006-11-01

There have been several reports of successful psychosocial interventions for children with selective mutism (SM), a disorder in which a child consistently fails to speak in one or more social settings (e.g., school) despite speaking normally in other settings (e.g., home). The present literature review was undertaken in order to provide an up-to-date summary and critique of the SM treatment literature published in the past fifteen years. PubMed, PsycINFO, and Web of Science databases were searched to identify SM treatment studies published in peer-reviewed journals between 1990 and 2005. A total of 23 studies were included in the present review. Of these, ten used a behavioral/cognitive behavioral approach, one used a behavioral language training approach, one used a family systems approach, five used a psychodynamic approach, and six used multimodal approaches to SM treatment. Although much of this literature is limited by methodological weaknesses, the existing research provides support for the use of behavioral and cognitive-behavioral interventions. Multimodal treatments also appear promising, but the essential components of these interventions have yet to be established. An outline of a cognitive-behavioral treatment package for a typical SM child is provided and the review concludes with suggestions for future research.

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.

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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

2014-03-01

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. © 2013 International Society of Neuropathology.

Atypical presentation of probable Creutzfeldt-Jakob disease associated with anti-Zic4 antibody: Literature review of neuronal antibodies in Creutzfeldt-Jakob disease.

Science.gov (United States)

Salazar, Richard

2018-05-01

Sporadic Creutzfeldt-Jakob disease is a prion disease characterized by rapidly progressive dementia, ataxia and myoclonus. Atypical phenotype masquerading as stroke, movement disorders or autoimmune encephalitis have been described. Here, I report a probable case of sCJD with an atypical presentation associated with anti-Zic4 antibody and review the literature of neuronal antibodies in CJD. A 70 year-old gentleman is admitted with a 2-month history of recurrent stroke-like symptoms associated with behavioral disturbances, gait ataxia and rapidly progressive dementia. His initial examination demonstrated akinetic mutism, diffuse rigidity, dysautononia, and Cheyne-Stokes respiration. Over the following weeks his condition progressed to profound coma. A comprehensive infectious, metabolic, inflammatory and autoimmune work-up yielded negative results. Empiric immunosuppressive therapy ensued. He expired three months after symptoms onset. Autopsy was not performed. After his demise, prion tests came back abnormal for elevated 14-3-3 protein, total tau and positive RTQuIC. Later on, anti-Zic4 antibodies were found in serum. This case underscores the importance of a high index of suspicion for CJD even in case of atypical features or the concurrence of neuronal antibodies. Further larger prospective studies on the prevalence of these neuronal antibodies in CJD and the contribution of these autoantibodies to disease pathophysiology are necessary. Copyright © 2018 Elsevier B.V. All rights reserved.

Selektívny mutizmus ako úzkostná porucha? Stručný prehľad o etiológii selektívneho mutizmu

OpenAIRE

Gabriela Majerechová

2015-01-01

Selective mutism is a disorder in which a child fluently speaks in well-known situations but is mute in less known situations. Despite overall etiology research, causes of mutism are still unrevealed. The paper summarizes various research studies which focused on links between selective mutism and anxiety disorders, particularly social phobia. In spite of some empirical evidence, it is still impossible to state that the selective mutism is an anxiety disorder.

Comparison of behavioral profiles for anxiety-related comorbidities including ADHD and selective mutism in children.

Science.gov (United States)

Levin-Decanini, Tal; Connolly, Sucheta D; Simpson, David; Suarez, Liza; Jacob, Suma

2013-09-01

Elucidating differences in social-behavioral profiles of children with comorbid presentations, utilizing caregiver as well as teacher reports, will refine our understanding of how contextual symptoms vary across anxiety-related disorders. In our pediatric anxiety clinic, the most frequent diagnoses and comorbidities were mixed anxiety (MA; ≥ 1 anxiety disorder; N = 155), anxiety with comorbid attention-deficit hyperactivity disorder (MA/ADHD, N = 47) and selective mutism (SM, N = 48). Behavioral measures (CPRS, CTRS) were analyzed using multiple one-way multivariate analyses of covariance tests. Differences between the three diagnostic groups were examined using completed parent and teacher reports (N = 135, 46, and 48 for MA, MA/ADHD, and SM groups, respectively). Comparisons across the MA, MA/ADHD, and SM groups indicate a significant multivariate main effect of group for caregiver and teacher responses (P < 0.01). Caregivers reported that children with SM are similar in profile to those with MA, and both groups were significantly different from the MA/ADHD group. Teachers reported that children with SM had more problems with social behaviors than with the MA or MA/ADHD groups. Further comparison indicates a significant main effect of group (P < 0.001), such that children with SM have the greatest differences in behavior observed by teachers versus caregivers. Clinical profiles between MA/ADHD, MA, and SM groups varied, illustrating the importance of multi-rater assessment scales to capture subtle distinctions and to inform treatment planning given that comorbidities occur frequently in children who present with anxiety. © 2013 Wiley Periodicals, Inc.

Application of a web-based cognitive-behavioural therapy programme for the treatment of selective mutism in Singapore: a case series study.

Science.gov (United States)

Ooi, Yoon Phaik; Raja, Malini; Sung, Sharon Cohan; Fung, Daniel S S; Koh, Jessie B K

2012-07-01

Selective mutism (SM) is characterised by limited or a lack of speech in selected social settings. Recent reviews suggest that cognitive-behavioural therapy (CBT) is an effective and promising treatment approach for SM. However, there is still a lack of studies documenting the applicability of CBT for SM in diverse populations. The goal of the present study was to examine the use of a web-based CBT programme ('Meeky Mouse') among Singaporean children diagnosed with SM. Five children with SM (one boy and four girls aged 6-11 years) participated in the 14-week 'Meeky Mouse' programme, in addition to being prescribed with an unchanged dosage of fluoxetine 10-20 mg daily. The progress made by the children throughout the course of the programme was documented by the therapist. Post treatment, four out of the five children demonstrated improvements in the frequency of speech during therapy sessions at home, in school and at other social situations. Findings from the present study provide support for the use of a web-based CBT programme in improving speech and decreasing the severity of SM among affected children.

Latent Class Symptom Profiles of Selective Mutism: Identification and Linkage to Temperamental and Social Constructs.

Science.gov (United States)

Diliberto, Rachele; Kearney, Christopher A

2017-11-21

Selective mutism (SM) is a stable, debilitating psychiatric disorder in which a child fails to speak in most public situations. Considerable debate exists as to the typology of this population, with empirically-based studies pointing to possible dimensions of anxiety, oppositionality, and communication problems, among other aspects. Little work has juxtaposed identified symptom profiles with key temperamental and social constructs often implicated in SM. The present study examined a large, diverse, non-clinical, international sample of children aged 6-10 years with SM to empirically identify symptom profiles and to link these profiles to key aspects of temperament (i.e., emotionality, shyness, sociability, activity) and social functioning (i.e., social problems, social competence). Exploratory and confirmatory factor analysis revealed anxiety/distress, oppositionality, and inattention domains. In addition, latent class analysis revealed nuanced profiles labeled as (1) moderately anxious, oppositional, and inattentive, (2) highly anxious, and moderately oppositional and inattentive, and (3) mildly to moderately anxious, and mildly oppositional and inattentive. Class 2 was the most impaired group and was associated with greater emotionality, shyness, and social problems. Class 3 was the least impaired group and was associated with better sociability and social competence and activity. Class 1 was largely between the other classes, demonstrating less shyness and social problems than Class 2. The results help confirm previous findings of anxiety and oppositional profiles among children with SM but that nuanced classes may indicate subtle variations in impairment. The results have implications not only for subtyping this population but also for refining assessment and case conceptualization strategies and pursuing personalized and perhaps less lengthy treatment.

Is selective mutism associated with deficits in memory span and visual memory?: An exploratory case-control study.

Science.gov (United States)

Kristensen, Hanne; Oerbeck, Beate

2006-01-01

Our main aim in this study was to explore the association between selective mutism (SM) and aspects of nonverbal cognition such as visual memory span and visual memory. Auditory-verbal memory span was also examined. The etiology of SM is unclear, and it probably represents a heterogeneous condition. SM is associated with language impairment, but nonspecific neurodevelopmental factors, including motor problems, are also reported in SM without language impairment. Furthermore, SM is described in Asperger's syndrome. Studies on nonverbal cognition in SM thus merit further investigation. Neuropsychological tests were administered to a clinical sample of 32 children and adolescents with SM (ages 6-17 years, 14 boys and 18 girls) and 62 nonreferred controls matched for age, gender, and socioeconomic status. We used independent t-tests to compare groups with regard to auditory-verbal memory span, visual memory span, and visual memory (Benton Visual Retention Test), and employed linear regression analysis to study the impact of SM on visual memory, controlling for IQ and measures of language and motor function. The SM group differed from controls on auditory-verbal memory span but not on visual memory span. Controlled for IQ, language, and motor function, the SM group did not differ from controls on visual memory. Motor function was the strongest predictor of visual memory performance. SM does not appear to be associated with deficits in visual memory span or visual memory. The reduced auditory-verbal memory span supports the association between SM and language impairment. More comprehensive neuropsychological studies are needed.

Relation between clinical findings and progression of cerebral cortical pathology in MM1-type sporadic Creutzfeldt-Jakob disease: proposed staging of cerebral cortical pathology.

Science.gov (United States)

Iwasaki, Yasushi; Tatsumi, Shinsui; Mimuro, Maya; Kitamoto, Tetsuyuki; Hashizume, Yoshio; Yoshida, Mari

2014-06-15

In our pathologic observation of the cerebral cortex including the neocortex, hippocampus, and limbic cortex in 43 Japanese patients with MM1-type sporadic Creutzfeldt-Jakob disease, the earliest pathologic finding was spongiform change and next was gliosis. Subsequently, neuropil rarefaction appeared, followed by neuron loss. On the basis of these observations, we propose the following cortical pathologic staging: Stage I, spongiform change; Stage II, hypertrophic astrocytosis; Stage III, neuropil rarefaction; Stage IV, neuron loss; Stage V, status spongiosus; and Stage VI, large cavity formation. We also suggest a more simple staging classification: Stages I and II, mild; Stages III and IV, moderate; and Stages V and VI, severe involvement. Based on statistical analysis of the cases, strong correlation coefficients were obtained between the neocortical and limbic pathologic stage and both total disease duration and brain weight. We estimated that the first observation times of cortical hyperintensity on diffusion-weighted images of magnetic resonance imaging, myoclonus, and periodic sharp wave complexes on the electroencephalogram approximately correspond to the early phase of Stage II of the neocortex. The time to reach the akinetic mutism state approximately corresponds to the middle phase of Stage II of the neocortex. Therefore, we think that approximate clinical manifestations at death, total disease duration, and brain weight can be estimated according to the pathologic stage of the neocortex or limbic cortex. Panencephalopathic-type pathology appeared approximately 12 months after disease onset, and this time approximately corresponds to the middle phase of Stage III of the neocortex. Copyright © 2014 Elsevier B.V. All rights reserved.

Sporadic Creutzfeldt-Jakob disease with focal findings: caveats to current diagnostic criteria

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Mader, Edward C.; El-Abassi, Rima; Villemarette-Pittman, Nicole R.; Santana-Gould, Lenay; Olejniczak, Piotr W.; England, John D.

2013-01-01

The clinical diagnosis of Creutzfeldt-Jakob disease (CJD) is largely based on the 1998 World Health Organization diagnostic criteria. Unfortunately, rigid compliance with these criteria may result in failure to recognize sporadic CJD (sCJD), especially early in its course when focal findings predominate and traditional red flags are not yet present. A 61-year-old man presented with a 3-week history of epilepsia partialis continua (jerking of the left upper extremity) and a 2-week history of forgetfulness and left hemiparesis; left hemisensory neglect was also detected on admission. Repeated brain magnetic resonance imaging (MRI) showed areas of restricted diffusion in the cerebral cortex, initially on the right but later spreading to the left. Electroence-phalography (EEG) on hospital days 7, 10, and 14 showed right-sided periodic lateralized epileptiform discharges. On day 20, the EEG showed periodic sharp wave complexes leading to a diagnosis of probable sCJD and subsequently to definite sCJD with brain biopsy. Neurological decline was relatively fast with generalized myoclonus and akinetic mutism developing within 7 weeks from the onset of illness. CJD was not immediately recognized because of the patient's focal/lateralized manifestations. Focal/lateralized clinical, EEG, and MRI findings are not uncommon in sCJD and EEG/MRI results may not be diagnostic in the early stages of sCJD. Familiarity with these caveats and with the most current criteria for diagnosing probable sCJD (University of California San Francisco 2007, MRI-CJD Consortium 2009) will enhance the ability to recognize sCJD and implement early safety measures. PMID:23717780

Increasing Verbal Behavior of a Student Who Is Selectively Mute

Science.gov (United States)

Beare, Paul; Torgerson, Colleen; Creviston, Cindy

2008-01-01

"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…

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.

Science.gov (United States)

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

2016-11-01

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.

Die terapeutiese gebruik van die scenotoets met verwysing na ...

African Journals Online (AJOL)

There is a paucity of applied research and available literature about selective mutism. The main aim of this study is to illustrate that the 'Scenotest', developed by Gerhild von Staabs (1991: 1), can be used as a therapeutic technique in treating children with selective mutism. A further aim is to supplement existing literature ...

A method for safely resecting anterior butterfly gliomas: the surgical anatomy of the default mode network and the relevance of its preservation.

Science.gov (United States)

Burks, Joshua D; Bonney, Phillip A; Conner, Andrew K; Glenn, Chad A; Briggs, Robert G; Battiste, James D; McCoy, Tressie; O'Donoghue, Daniel L; Wu, Dee H; Sughrue, Michael E

2017-06-01

OBJECTIVE Gliomas invading the anterior corpus callosum are commonly deemed unresectable due to an unacceptable risk/benefit ratio, including the risk of abulia. In this study, the authors investigated the anatomy of the cingulum and its connectivity within the default mode network (DMN). A technique is described involving awake subcortical mapping with higher attention tasks to preserve the cingulum and reduce the incidence of postoperative abulia for patients with so-called butterfly gliomas. METHODS The authors reviewed clinical data on all patients undergoing glioma surgery performed by the senior author during a 4-year period at the University of Oklahoma Health Sciences Center. Forty patients were identified who underwent surgery for butterfly gliomas. Each patient was designated as having undergone surgery either with or without the use of awake subcortical mapping and preservation of the cingulum. Data recorded on these patients included the incidence of abulia/akinetic mutism. In the context of the study findings, the authors conducted a detailed anatomical study of the cingulum and its role within the DMN using postmortem fiber tract dissections of 10 cerebral hemispheres and in vivo diffusion tractography of 10 healthy subjects. RESULTS Forty patients with butterfly gliomas were treated, 25 (62%) with standard surgical methods and 15 (38%) with awake subcortical mapping and preservation of the cingulum. One patient (1/15, 7%) experienced postoperative abulia following surgery with the cingulum-sparing technique. Greater than 90% resection was achieved in 13/15 (87%) of these patients. CONCLUSIONS This study presents evidence that anterior butterfly gliomas can be safely removed using a novel, attention-task based, awake brain surgery technique that focuses on preserving the anatomical connectivity of the cingulum and relevant aspects of the cingulate gyrus.

[A case of MM1+2 Creutzfeldt-Jakob disease with a longitudinal study of EEG and MRI].

Science.gov (United States)

Katsube, Mizuho; Shiota, Yuri; Harada, Takayuki; Shibata, Hiroshi; Nagai, Atsushi

2013-11-01

We report a case of definite MM1 + 2 sporadic Creutzfeldt-Jakob disease (sCJD). A 66-year-old woman was admitted to our hospital with memory disturbance and disorientation for three months. On admission she presented a progressive cognitive insufficiency. Electroencephalography (EEG) revealed a frontal intermittent rhythmical delta activity (FIRDA) and the brain magnetic resonance imaging (MRI) showed high signal intensities in cerebral cortex on diffusion weighted images (DWI). After four months from the onset, she reached the akinetic mutism state followed by myoclonus. Follow up examination revealed that periodic synchronous discharge (PSD) was found in EEG, and DWI revealed enlargement of high signal intensity lesions in cerebral cortex. At seven months from the onset, PSD and high signal intensities of cortex became unclear with disappearance of myoclonus, and brain white matter lesions were evident on MRI. Serial studies of EEG and MRI revealed that PSD generalized from frontal lobe dominant pattern, while high signal intensity lesions of cortex diffusely increased on DWI. At ten months from the onset patient died. Pathological examination in brain showed moderate and diffuse neuronal cell loss and gliosis in cerebral cortex corresponding with DWI changes. The genotype at codon 129 of the prion protein (PrP) was homozygous methionine (MM) and the type of protease-resistant PrP (PrPres) was the mixed type of 1 and 2 in Western blot analysis. It has been rare to analyze the changes of EEG and MRI in the entire stage and to investigate pathological finding in the case of sCJD-MM1 + 2. A longitudinal examination of EEG and MRI is useful for early diagnosis of CJD. Also we could correlate these findings with clinical and histopathological phenotype.

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

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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

1999-07-01

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

Sporadic Creutzfeldt-Jakob disease with unusual initial presentation as posterior reversible encephalopathy syndrome: a case report.

Science.gov (United States)

Dirzius, Edgaras; Balnyte, Renata; Steibliene, Vesta; Gleizniene, Rymante; Gudinaviciene, Inga; Radziunas, Andrius; Petrikonis, Kestutis

2016-11-22

Creutzfeldt - Jakob disease (CJD) is a rapidly progressive and fatal neurodegenerative prion disease. MRI findings are included in diagnostic criteria for probable CJD, giving a sensitivity and specificity more than 90%, but the atypical radiological presentations in the early stage of the disease could cause the diagnostic difficulties. CJD can be definitively diagnosed by histopathological confirmation, brain biopsy or at autopsy. We present a case of 53-year-old woman with a history of a rapidly progressive dementia with symptoms of visual impairment, increased extrapyramidal type muscle tonus, stereotypical movements and ataxic gait resulting in the patient's death after13 months. The clinical symptoms deteriorated progressively to myoclonus and akinetic mutism already on the 14th week. The series of diagnostic examinations were done to exclude the possible causes of dementia. Initial MRI evaluation as posterior reversible encephalopathy syndrome (PRES) on the 9th week after the onset of symptoms created us a diagnostic conundrum. Subsequent MRI findings of symmetrical lesions in the basal ganglia (nucleus caudatus, putamen) on the 13th week and EEG with periodic sharp wave complexes (PSWC) in frontal regions on the 18th week allowed us to diagnose the probable sCJD. The histopathological findings after brain biopsy on the 14th week demonstrated the presence of the abnormal prion protein deposits in the grey matter by immunohistochemistry with ICSM35, KG9 and 12 F10 antibodies and confirmed the diagnosis of sCJD. In this article we focus our attention on a rare association between radiological PRES syndrome and early clinical stage of sCJD. Although concurrent manifestation of these conditions can be accidental, but the immunogenic or neuropeptide mechanisms could explain such radiological MRI findings. A thorough knowledge of differential diagnostic of PRES may be especially useful in earlier diagnosis of sCJD.

Novel mutation of the PRNP gene of a clinical CJD case

Directory of Open Access Journals (Sweden)

Collinge John

2006-11-01

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.

A common genetic variant in the neurexin superfamily member CNTNAP2 is associated with increased risk for selective mutism and social anxiety-related traits.

Science.gov (United States)

Stein, Murray B; Yang, Bao-Zhu; Chavira, Denise A; Hitchcock, Carla A; Sung, Sharon C; Shipon-Blum, Elisa; Gelernter, Joel

2011-05-01

Selective mutism (SM), considered an early-onset variant of social anxiety disorder, shares features of impaired social interaction and communication with autism spectrum disorders (ASDs) suggesting a possible shared pathophysiology. We examined association of a susceptibility gene, contactin-associated protein-like 2 (CNTNAP2), for ASDs and specific language impairment with SM and social anxiety-related traits. Sample 1 subjects were 99 nuclear families including 106 children with SM. Sample 2 subjects were young adults who completed measures of social interactional anxiety (n = 1028) and childhood behavioral inhibition (n = 920). Five single nucleotide polymorphisms in CNTNAP2 (including rs7794745 and rs2710102, previously associated with ASDs) were genotyped. Analyses revealed nominal significance (p = .018) for association of SM with rs2710102, which, with rs6944808, was part of a common haplotype associated with SM (permutation p = .022). Adjusting for sex and ancestral proportion, each copy of the rs2710102*a risk allele in the young adults was associated with increased odds of being >1 SD above the mean on the Social Interactional Anxiety Scale (odds ratio = 1.33, p = .015) and Retrospective Self-Report of Inhibition (odds ratio = 1.40, p = .010). Although association was found with rs2710102, the risk allele (a) for the traits studied here is the nonrisk allele for ASD and specific language impairment. These findings suggest a partially shared etiology between ASDs and SM and raise questions about which aspects of these syndromes are potentially influenced by CNTNAP2 and mechanism(s) by which these influences may be conveyed. Copyright © 2011 Society of Biological Psychiatry. Published by Elsevier Inc. All rights reserved.

STUDY ABOUT THE INCIDENCE OF HEARING-SPEAKING DISORDERS IN A POPULATION WITH MENTAL DEFICIENCY

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Ioana Mihaela Tomulescu

2003-01-01

Full Text Available This study is about the incidence of hearing-speaking disorders in a population with mental deficiency. We studied 596 children interned in Neurology and Psychiatry Clinical Hospital of Oradea during the 1999 - 2001 period. In 596 children, 393 presented different types of mental deficiency. The most frequent disorders observed are hearing loss or deafness, deaf-mutism, mutism and speaking retardation. Also, we related an increased frequency in rural area and in group of children with severe mental deficiency.

Characterization and in vivo regulon determination of an ECF sigma factor and its cognate anti-sigma factor in Nostoc punctiforme.

Science.gov (United States)

Bell, Nicole; Lee, Jamie J; Summers, Michael L

2017-04-01

Based on primary sequence comparisons and genomic context, Npun_F4153 (SigG)/Npun_F4154 (SapG) of the cyanobacterium Nostoc punctiforme were hypothesized to encode an ECF sigma factor/anti-sigma factor pair. Transcription of sigG increased in heterocysts and akinetes, and after EDTA treatment. Interaction between SigG and the predicted cytoplasmic domain of SapG was observed in vitro. A SigG-GFP translational fusion protein localized to the periphery of vegetative cells in vivo, but lost this association following heat stress. A sigG mutant was unable to survive envelope damage caused by heat or EDTA, but was able to form functional heterocysts. Akinetes in the mutant strain appeared normal, but these cultures were less resistant to lysozyme and cold treatments than those of the wild-type strain. The SigG in vivo regulon was determined before and during akinete differentiation using DNA microarray analysis, and found to include multiple genes with putative association to the cell envelope. Mapped promoters common to both arrays enabled identification of a SigG promoter-binding motif that was supported in vivo by reporter studies, and in vitro by run-off transcription experiments. These findings support SigG/SapG as a sigma/anti-sigma pair involved in repair of envelope damage resulting from exogenous sources or cellular differentiation. © 2017 John Wiley & Sons Ltd.

An autopsied case of MM1 + MM2-cortical with thalamic-type sporadic Creutzfeldt-Jakob disease presenting with hyperintensities on diffusion-weighted MRI before clinical onset.

Science.gov (United States)

Iwasaki, Yasushi; Mori, Keiko; Ito, Masumi; Mimuro, Maya; Kitamoto, Tetsuyuki; Yoshida, Mari

2017-02-01

A 78-year-old Japanese man presented with rapidly progressive dementia and gait disturbances. Eight months before the onset of clinical symptoms, diffusion-weighted magnetic resonance imaging (DWI) demonstrated hyperintensities in the right temporal, right parietal and left medial occipital cortices. Two weeks after symptom onset, DWI showed extensive hyperintensity in the bilateral cerebral cortex, with regions of higher brightness that existed prior to symptom onset still present. Four weeks after clinical onset, periodic sharp wave complexes were identified on an electroencephalogram. Myoclonus was observed 8 weeks after clinical onset. The patient reached an akinetic mutism state and died 5 months after onset. Neuropathological examination showed widespread cerebral neocortical involvement of fine vacuole-type spongiform changes with large confluent vacuole-type spongiform changes. Spongiform degeneration with neuron loss and hypertrophic astrocytosis was also observed in the striatum and medial thalamus. The inferior olivary nucleus showed severe neuron loss with hypertrophic astrocytosis. Prion protein (PrP) immunostaining showed widespread synaptic-type PrP deposition with perivacuolar-type PrP deposition in the cerebral neocortex. Mild to moderate PrP deposition was also observed extensively in the basal ganglia, thalamus, cerebellum and brainstem, but it was not apparent in the inferior olivary nucleus. PrP gene analysis showed no mutations, and polymorphic codon 129 showed methionine homozygosity. Western blot analysis of protease-resistant PrP showed both type 1 scrapie type PrP (PrP Sc ) and type 2 PrP Sc . Based on the relationship between the neuroimaging and pathological findings, we speculated that cerebral cortical lesions with large confluent vacuoles and type 2 PrP Sc would show higher brightness and continuous hyperintensity on DWI than those with fine vacuoles and type 1 PrP Sc . We believe the present patient had a combined form of MM1�

Growth Characteristics of an Estuarine Heterocystous Cyanobacterium

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Pablo Guimarães

2017-06-01

Full Text Available A new estuarine filamentous heterocystous cyanobacterium was isolated from intertidal sediment of the Lagoa dos Patos estuary (Brazil. The isolate may represent a new genus related to Cylindrospermopsis. While the latter is planktonic, contains gas vesicles, and is toxic, the newly isolated strain is benthic and does not contain gas vesicles. It is not known whether the new strain is toxic. It grows equally well in freshwater, brackish and full salinity growth media, in the absence of inorganic or organic combined nitrogen, with a growth rate 0.6 d-1. Nitrogenase, the enzyme complex responsible for fixing dinitrogen, was most active during the initial growth phase and its activity was not different between the different salinities tested (freshwater, brackish, and full salinity seawater. Salinity shock also did not affect nitrogenase activity. The frequency of heterocysts was high, coinciding with high nitrogenase activity during the initial growth phase, but decreased subsequently. However, the frequency of heterocysts decreased considerably more at higher salinity, while no change in nitrogenase activity occurred, indicating a higher efficiency of dinitrogen fixation. Akinete frequency was low in the initial growth phase and higher in the late growth phase. Akinete frequency was much lower at high salinity, which might indicate better growth conditions or that akinete differentiation was under the same control as heterocyst differentiation. These trends have hitherto not been reported for heterocystous cyanobacteria but they seem to be well fitted for an estuarine life style.

Basal hyperaemia is the primary abnormality of perfusion in Takotsubo cardiomyopathy

DEFF Research Database (Denmark)

Christensen, Thomas Emil; Ahtarovski, Kiril Aleksov; Bang, Lia Evi

2015-01-01

AIMS: Takotsubo cardiomyopathy (TTC) is characterized by acute completely reversible regional left ventricle (LV) akinesia and decreased tracer uptake in the akinetic region on semi-quantitative perfusion imaging. The latter may be due to normoperfusion of the akinetic mid/apical area and basal...... hyperperfusion. Our aim was to examine abnormalities of perfusion in TTC, and we hypothesized that basal hyperperfusion is the primary perfusion abnormality in the acute state. METHOD AND RESULTS: Twenty-five patients were diagnosed with TTC due to (i) acute onset of symptoms, (ii) typical apical ballooning......-on follow-up. Patients initially had severe heart failure, mid/apical oedema but no infarction, and a rise in cardiac biomarkers. On initial perfusion PET imaging, eight patients appeared to have normal, whereas 17 patients had impaired LV perfusion. In the latter, flow in the basal region was increased...

First report of an Anabaena Bory strain containing microcystin-LR in a freshwater body in Africa

CSIR Research Space (South Africa)

Oberholster, Paul J

2015-03-11

Full Text Available algae belonged to the genus Anabaena, a family of filamentous cyanobacteria known to produce cyanotoxins such as anatoxin-a, harmful to humans and the aquatic foodweb. The specimens isolated lacked the characteristic akinetes and/or heterocysts...

Motor phenotype and magnetic resonance measures of basal ganglia iron levels in Parkinson's disease.

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Bunzeck, Nico; Singh-Curry, Victoria; Eckart, Cindy; Weiskopf, Nikolaus; Perry, Richard J; Bain, Peter G; Düzel, Emrah; Husain, Masud

2013-12-01

In Parkinson's disease the degree of motor impairment can be classified with respect to tremor dominant and akinetic rigid features. While tremor dominance and akinetic rigidity might represent two ends of a continuum rather than discrete entities, it would be important to have non-invasive markers of any biological differences between them in vivo, to assess disease trajectories and response to treatment, as well as providing insights into the underlying mechanisms contributing to heterogeneity within the Parkinson's disease population. Here, we used magnetic resonance imaging to examine whether Parkinson's disease patients exhibit structural changes within the basal ganglia that might relate to motor phenotype. Specifically, we examined volumes of basal ganglia regions, as well as transverse relaxation rate (a putative marker of iron load) and magnetization transfer saturation (considered to index structural integrity) within these regions in 40 individuals. We found decreased volume and reduced magnetization transfer within the substantia nigra in Parkinson's disease patients compared to healthy controls. Importantly, there was a positive correlation between tremulous motor phenotype and transverse relaxation rate (reflecting iron load) within the putamen, caudate and thalamus. Our findings suggest that akinetic rigid and tremor dominant symptoms of Parkinson's disease might be differentiated on the basis of the transverse relaxation rate within specific basal ganglia structures. Moreover, they suggest that iron load within the basal ganglia makes an important contribution to motor phenotype, a key prognostic indicator of disease progression in Parkinson's disease. Copyright © 2013 The Authors. Published by Elsevier Ltd.. All rights reserved.

Morphological traits in nitrogen fixing heterocytous cyanobacteria: possible links between morphology and eco-physiology.

Czech Academy of Sciences Publication Activity Database

Pinto, P. D.; Kust, Andreja; Devercelli, M.; Kozlíková-Zapomělová, Eliška

2016-01-01

Roč. 764, č. 1 (2016), s. 271-281 ISSN 0018-8158 R&D Projects: GA ČR(CZ) GA14-18067S Institutional support: RVO:60077344 Keywords : traits * heterocyte * akinete * shape * size * phytoplankton Subject RIV: DA - Hydrology ; Limnology Impact factor: 2.056, year: 2016

Phylogenetic analysis of the genus Anabaena based on PCR ...

African Journals Online (AJOL)

In this study, ten species of Anabaena were used to test the congruence between the traditional morphological classification system and the present molecular classification system. For morphological classification, strains were categorized into two different groups based on the whether or not the akinetes were directly ...

African Journal of Neurological Sciences - 2009 Vol. 28 No 1

African Journals Online (AJOL)

Postoperative mutism is an infrequent complication of brain surgery. .... occurs in individuals with mixed cerebral dominance for language function. ... It is possible that in some right-handed patients who develop left cerebral dysfunction, ...

Mode of presentation of patients of dissociative (conversion) disorder at the armed forces institute of mental health

International Nuclear Information System (INIS)

Jan, A.U.; Jehangir, S.

2015-01-01

To determine the mode of presentation of dissociative disorders presenting at Armed Forces Institute of Mental Health. Study Design: Cross sectional study. Place and Duration of Study: The study was conducted at the Armed Forces Institute of Mental Health (AFIMH) Rawalpindi from 1st June 2013 to 31st August 2013. Patients and Methods: Fifty four patients of dissociative disorders were included in the study by using consecutive non-probability sampling. Category of presentation of dissociative disorders in the participants was determined by the primary mode of presentation and by using international classification of diseases (ICD)-10 diagnostic guidelines. Results: The commonest type of presentation of dissociative disorders was mutism (40.7%), possession state (18.5%), pseudo fits (12.9%) followed by paraparesis (9.2%). Conclusion: Predominantly the patients presented with mutism (dissociative motor disorder). (author)

Delayed cerebral ischemia after aneurysmal subarachnoid hemorrhage: clinicoanatomic correlations

NARCIS (Netherlands)

Hijdra, A.; van Gijn, J.; Stefanko, S.; van Dongen, K. J.; Vermeulen, M.; van Crevel, H.

1986-01-01

Fifty-seven of 176 prospectively studied patients with aneurysmal subarachnoid hemorrhage (SAH) developed delayed cerebral ischemia. Clinical features included hemispheric focal signs (13), decrease in level of consciousness (14), or both (30), and mutism (15). Forty-seven patients showed hypodense

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.

2014-01-01

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

Cannabis Induced Periodic Catatonia: A Case Report

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

2011-01-01

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…

Examination of Urinary Iodine Status from a Sample of Malian ...

African Journals Online (AJOL)

In pregnant women, this deficiency can lead to congenital hypothyroidism in newborn infants. Congenital hypothyroidism is a disorder associated with a low IQ, deaf mutism, and neurological problems. Many studies have revealed that iodine deficiency is common throughout West Africa. While several of these studies have ...

Symbolization Levels in Communicative Behaviors of Children Showing Pervasive Developmental Disorders.

Science.gov (United States)

Atlas, Jeffrey A.; Lapidus, Leah Blumberg

1988-01-01

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…

Auditory-perceptual speech analysis in children with cerebellar tumours: a long-term follow-up study.

Science.gov (United States)

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

2012-09-01

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. Copyright © 2012 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

His Trail of Silence: A Case Study in Reaching a Child Who Refused To Speak.

Science.gov (United States)

Van Antwerp, Kathleen

1999-01-01

Discusses selective mutism, a childhood disorder characterized by persistent failure to speak in specific social situations. Details a case study of one boy from kindergarten until treatment was received in 8th grade. Discusses techniques used in the communication process between the boy and his counselor. (Author/JDM)

Intervention with the Selectively Mute Child.

Science.gov (United States)

Porjes, Michelle D.

1992-01-01

Defines selective mutism as describing children who actively choose to speak to few people in selected environments, noting it is most commonly used to describe nonverbal behavior in school setting. Reviews literature from psychoanalytic and learning theory approaches. Presents intervention strategies used with two selectively mute first graders.…

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

Science.gov (United States)

Bebko, James M.

1990-01-01

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…

Establishing Vocal Verbalizations in Mute Mongoloid Children.

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Buddenhagen, Ronald G.

Behavior modification as an attack upon the problem of mutism in mongoloid children establishes the basis of the text. Case histories of four children in a state institution present the specific strategy of speech therapy using verbal conditioning. Imitation and attending behavior, verbal chaining, phonetic theory, social reinforcement,…

Narrative Counseling for Professional School Counselors

Science.gov (United States)

Nafziger, Jacinta; DeKruyf, Lorraine

2013-01-01

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…

Iodine deficiency, thyroid function and hearing deficit: a review

NARCIS (Netherlands)

Boonstra, A.; MacKenzie, I.

2013-01-01

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

Factors controlling induction of reproduction in algae--review: the text.

Science.gov (United States)

Agrawal, S C

2012-09-01

This review surveys on the influence of different environmental factors like light (intensity, quality, photoperiod), temperature, season, nutrients (inorganic, organic), biotic factors (algal extracellular products, bacterial association, animals grazing), osmotic stress, pH of the medium, wave motion and mechanical shock, pollution, and radiations (UV, X-rays, gamma radiation) on the induction (or inhibition) of algal reproduction like cell division in unicellular algae, and formation of zoospores, aplanospores, akinetes, cysts, antheridia, oogonia, zygospores, etc.

Interviews with Selectively Mute Children

Science.gov (United States)

Omdal, Heidi; Galloway, David

2007-01-01

The assessment of selective mutism usually takes place in a clinic, where the child often refuses to speak to the therapist. The challenge when trying to understand the child's own perspective is to find a medium for communication. Three selectively mute children were interviewed using Raven's Controlled Projection for Children (RCPC). The…

Role of Cerebellum in Fine Speech Control in Childhood: Persistent Dysarthria after Surgical Treatment for Posterior Fossa Tumour

Science.gov (United States)

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

2011-01-01

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…

Accountability Steps for Highly Reluctant Speech: Tiered-Services Consultation in a Head Start Classroom

Science.gov (United States)

Howe, Heather; Barnett, David

2013-01-01

This consultation description reports parent and teacher problem solving for a preschool child with no typical speech directed to teachers or peers, and, by parent report, normal speech at home. This child's initial pattern of speech was similar to selective mutism, a low-incidence disorder often first detected during the preschool years, but…

Delayed Diagnosis in an Elderly Schizophrenic Patient with Catatonic State and Pulmonary Embolism

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Hsueh-Chin Hu

2013-09-01

Full Text Available Catatonia is a syndrome with any two of five core features: stupor/motoric immobility/catalepsy/waxy flexibility, excitement, negativism/mutism, posturing, and echolalia/echopraxia. We describe a case of delayed diagnosis of pulmonary embolism with an atypical presentation in an elderly schizophrenia male patient, which led to a life-threatening brain infarction. A 75-year-old male was hospitalized in a psychiatric ward because of stupor, poor intake and mutism under a diagnosis of recurrent catatonia. His inability to express his suffering, dehydration, exacerbation of chronic obstructive pulmonary disease, and upper gastrointestinal bleeding, however, made an accurate diagnosis difficult. Finally, the high D-dimer level and further chest computed tomography confirmed pulmonary embolism in the trunk of the bilateral main pulmonary arteries. The brain computed tomography also confirmed brain infarcts. He was transferred to the cardiac intensive care unit and was eventually rescued from near death due to pulmonary embolism and brain infarction. A careful differential diagnosis for pulmonary embolism-induced delirium and catatonic state is important in the treatment of patients with a previous diagnosis of catatonic schizophrenia.

Pseudobulbar dysarthria in the initial stage of motor neuron disease with dementia: a clinicopathological report of two autopsied cases.

Science.gov (United States)

Ishihara, Kenji; Araki, Shigeo; Ihori, Nami; Suzuki, Yoshio; Shiota, Jun-ichi; Arai, Nobutaka; Nakano, Imaharu; Kawamura, Mitsuru

2013-01-01

We retrospectively analyzed the clinical features of two cases of neurodegenerative disease, whose initial symptoms were motor speech disorder and dementia, brought to autopsy. We compared the distributions of pathological findings with the clinical features. The main symptom of speech disorder was dysarthria, involving low pitch, slow rate, hypernasality and hoarseness. Other than these findings, effortful speech, sound prolongation and initial difficulty were observed. Moreover, repetition of multisyllables was severely impaired compared to monosyllables. Repetition and comprehension of words and sentences were not impaired. Neither atrophy nor fasciculation of the tongue was observed. Both cases showed rapid progression to mutism within a few years. Neuropathologically, frontal lobe degeneration including the precentral gyrus was observed. The bilateral pyramidal tracts also showed severe degeneration. However, the nucleus of the hypoglossal nerve showed only mild degeneration. These findings suggest upper motor neuron dominant motor neuron disease with dementia. We believe the results indicate a subgroup of motor neuron disease with dementia whose initial symptoms involve pseudobulbar palsy and dementia, and which shows rapid progression to mutism. Copyright © 2013 S. Karger AG, Basel.

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African Journals Online (AJOL)

2004-03-30

Mar 30, 2004 ... and to obtain selectivity for the determination of iodine in quirement for iodine is 1 - 2 mg/kg of body weight ... ism; characterized by mental deficiency, deaf mutism and possible. Glass distilled water was used ... rachloride with a clean up based on a selective redox-strip-. Pathological histories like age of the ...

Afraid To Be Heard: The Selectively Mute Child.

Science.gov (United States)

Longo, Sharon L.

2001-01-01

Presents facts about selective mutism, an anxiety disorder believed to be caused by low levels of serotonin in the brain, discussing its effects on school children, explaining how to get the necessary help (e.g., talking to health professionals and becoming educated about the disorder), and noting what parents can do (e.g., help raise the child's…

Transient Stuttering in Catatonic Bipolar Patients

Directory of Open Access Journals (Sweden)

Anthony B. Joseph

1991-01-01

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.

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

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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.

Without a Voice

Science.gov (United States)

Pennamacoor, Carol

2007-01-01

Selective Mutism (SM), normally discovered at a young age, is a selective anxiety disorder in which a child demonstrates a persistent lack of speech in one or more social settings, notably in school, despite his or her ability to speak comfortably at home. In this article, the author presents Betsy, a child who was diagnosed with SM and was helped…

Can Autism Spectrum Disorders and Social Anxiety Disorders Be Differentiated by the Social Responsiveness Scale in Children and Adolescents?

Science.gov (United States)

Cholemkery, Hannah; Mojica, Laura; Rohrmann, Sonja; Gensthaler, Angelika; Freitag, Christine M.

2014-01-01

Autism spectrum disorder (ASD) as well as social phobia (SP), and selective mutism (SM) are characterised by impaired social interaction. We assessed the validity of the Social Responsiveness Scale (SRS) to differentiate between ASD, and SP/SM. Raw scores were compared in 6-18 year old individuals with ASD (N = 60), SP (N = 38), SM (N = 43), and…

Frontotemporal dementia and neurocysticercosis: a case report

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Corina Satler

Full Text Available ABSTRACT We report a case of a 67-year-old woman with frontotemporal dementia (FTD and a history of neurocysticercosis. After her retirement she showed progressive behavioral changes and neuropsychiatric symptoms with relative preservation of cognitive functioning. During the next three years, the patient manifested progressive deterioration of verbal communication gradually evolving to mutism, a hallmark of cases of progressive nonfluent aphasia.

Coronary collateral vessels in patients with previous myocardial infarction

International Nuclear Information System (INIS)

Nakatsuka, M.; Matsuda, Y.; Ozaki, M.

1987-01-01

To assess the degree of collateral vessels after myocardial infarction, coronary angiograms, left ventriculograms, and exercise thallium-201 myocardial scintigrams of 36 patients with previous myocardial infarction were reviewed. All 36 patients had total occlusion of infarct-related coronary artery and no more than 70% stenosis in other coronary arteries. In 19 of 36 patients with transient reduction of thallium-201 uptake in the infarcted area during exercise (Group A), good collaterals were observed in 10 patients, intermediate collaterals in 7 patients, and poor collaterals in 2 patients. In 17 of 36 patients without transient reduction of thallium-201 uptake in the infarcted area during exercise (Group B), good collaterals were seen in 2 patients, intermediate collaterals in 7 patients, and poor collaterals in 8 patients (p less than 0.025). Left ventricular contractions in the infarcted area were normal or hypokinetic in 10 patients and akinetic or dyskinetic in 9 patients in Group A. In Group B, 1 patient had hypokinetic contraction and 16 patients had akinetic or dyskinetic contraction (p less than 0.005). Thus, patients with transient reduction of thallium-201 uptake in the infarcted area during exercise had well developed collaterals and preserved left ventricular contraction, compared to those in patients without transient reduction of thallium-201 uptake in the infarcted area during exercise. These results suggest that the presence of viable myocardium in the infarcted area might be related to the degree of collateral vessels

IMPULSIVE-COMPULSIVE DISORDERS IN PARKINSON'S DISEASE. CLINICAL CASES

OpenAIRE

N. V. Fedorova; A. V. Nikitina

2015-01-01

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...

Sedimentation and occurrence and trends of selected nutrients, other chemical constituents, and cyanobacteria in bottom sediment, Clinton Lake, northeast Kansas, 1977-2009

Science.gov (United States)

Juracek, Kyle E.

2011-01-01

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

Silent at school--elective mutism and abuse.

Science.gov (United States)

MacGregor, R; Pullar, A; Cundall, D

1994-01-01

A retrospective case-control study of electively mute children from one city is reported. Eight of 18 children selectively mute in school had suffered definite or probable abuse compared with only one control with a speech or language problem, and no classroom controls. The implications for management are discussed. PMID:8048829

Transient mutism after anterior transcallosal approach | Naama ...

African Journals Online (AJOL)

African Journal of Neurological Sciences. Journal Home · ABOUT THIS JOURNAL · Advanced Search · Current Issue · Archives · Journal Home > Vol 34, No 1 (2015) >. Log in or Register to get access to full text downloads.

Differential Diagnosis and Management of Incomplete Locked-In Syndrome after Traumatic Brain Injury

Directory of Open Access Journals (Sweden)

Lauren Surdyke

2017-01-01

Full Text Available Locked-in syndrome (LIS is a rare diagnosis in which patients present with quadriplegia, lower cranial nerve paralysis, and mutism. It is clinically difficult to differentiate from other similarly presenting diagnoses with no standard approach for assessing such poorly responsive patients. The purpose of this case is to highlight the clinical differential diagnosis process and outcomes of a patient with LIS during acute inpatient rehabilitation. A 32-year-old female was admitted following traumatic brain injury. She presented with quadriplegia and mutism but was awake and aroused based on eye gaze communication. The rehabilitation team was able to diagnose incomplete LIS based on knowledge of neuroanatomy and clinical reasoning. Establishing this diagnosis allowed for an individualized treatment plan that focused on communication, coping, family training, and discharge planning. The patient was ultimately able to discharge home with a single caregiver, improving her quality of life. Continued evidence highlights the benefits of intensive comprehensive therapy for those with acquired brain injury such as LIS, but access is still limited for those with a seemingly poor prognosis. Access to a multidisciplinary, specialized team provides opportunity for continued assessment and individualized treatment as the patient attains more medical stability, improving long-term management.

Chronic catatonia with obsessive compulsive disorder symptoms treated with lorazepam, memantine, aripiprazole, fluvoxamine and neurosurgery

OpenAIRE

Mukai, Yuki; Two, Aimee; Jean-Baptiste, Michel

2011-01-01

Catatonia is a syndrome with protean manifestations and multiple aetiologies. In this report, the authors describe the case of a young woman who presented for care after a 13-year period of catatonia-like symptoms, including mutism, refusal to eat and persistent neck flexion. Medical management included placement of a percutaneous endoscopic gastric tube for nutritional support. A thorough medical investigation later revealed the presence of a cervical spine haemangioma that was treated surgi...

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

2008-04-01

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

Assessment of relationship between regional perfusion and ventricular function in patients with severely depressed ejection fraction

International Nuclear Information System (INIS)

Teresinska, A.; Konieczna, S.; Szumilak, B.; Gosiewska-Marcinkowska, E.; Potocka, J.

1998-01-01

Patients with low ventricular (LV) ejection fraction (EF) and with regional or global LV dysfunction, considered for myocardial revascularization, are often submitted to myocardial perfusion study to ptrove perfusion preserved and to approximate viability. The aim of this work is to evaluate, to what extent SPECT with Tc-99m-MIBI (SPECT-MIBI) additionally differentiatesa and enlarges the information on LV contractility achieved from radioisotopic ventriculography (RNV). Seventy-three patients with EF=0.11-0.35 (mean: 0.26 ± 0.06, calculated from RNV) were studied. Planar gated RNV and SPECT-MIBI were performed within 2 months (mean: 15 ± 14 days). RNV, after in vivo red cells labeling with Tc-99m, was recorded in rest in LAO45 and RAO30 views. Global EF and contractility of 5 regions (anterior, posterior, lateral walls, septum and apex) were evaluated. Assessment was performed by means of LV in 'cine' mode,m ED and ES outlines and amplitude-phase images. Regional contractility abnormalities were classified as dyskinesis, akinesis and hypokinesis. SPECT-MIBI was performed in rest and stress. Perfusion abnormalities in 5 above mentioned regions was classified as large (more intense than 50% of myocardial maximum) or small persistent defects (DEFpers), large or small partially reversible defects (DEFpart-rev), large or small completely reversible defects (DEFrev). Hypokinesis was detected in 23-35 regions (65%) , dyskinesis - in 63 (17%), akinesis - in 37 (10%), normokinesis - in 27 (7%). In dyskinetic regions, there existed large DEFpers (79%) or large DEFpart-rev (21%). In akinetic regions, there also existed large DEFpers (68%) or large DEFpart-rev (32%). In hypokinetic regions, there were no perfusion defects (31% of regions), small DEFpers, DEFrev and DEFpart-rev (42%) and also large DEFpers (27%). In most of normokinetic regions, different types of perfusion defects were observed (large DEFpers existed in 26% of regions). In summary: In dyskinetic and akinetic

Serotonin syndrome associated with sertraline use: case report

OpenAIRE

Bárbara Werner Griciunas; Norton Yoshiaki Kitanishi; Renata Carvalho de Souza; Daniel Azevedo Cavalcante; Leonardo Mattiolli Marini

2017-01-01

Case report of serotonin syndrome in patient who initiated the use of sertraline at a dose greater than twice the recommended for the treatment of psychotic depression. The patient presented contracture of the limbs, puzzled look, mutism and blood pressure 230x110 mmHg. The syndrome is increasingly common, although it is not well recognized. Many medications can cause it and this possibility should be considered in patients taking serotonergic drugs presenting autonomic or mental disorders an...

The 7q11.23 Microduplication Syndrome: A Clinical Report with Review of Literature

OpenAIRE

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

2016-01-01

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 deve...

The Youth Anxiety Measure for DSM-5 (YAM-5): Correlations with anxiety, fear, and depression scales in non-clinical children.

Science.gov (United States)

Muris, Peter; Mannens, Janne; Peters, Lisanne; Meesters, Cor

2017-10-01

The Youth Anxiety Measure for DSM-5 (YAM-5) is a newly developed rating scale for assessing anxiety disorder symptoms of children and adolescents in terms of the contemporary classification system. In the present study, 187 children aged 8-12 years completed the new measure as well as the trait version of the State-Trait Anxiety Inventory for Children (STAIC), the Short Form of the Fear Survey Schedule for Children-Revised (FSSC-R-SF), the Spence Children's Anxiety Scale (SCAS), the Selective Mutism Questionnaire (SMQ), and the Children's Depression Inventory (CDI). Results indicated that part one of the YAM-5, which measures symptoms of the major anxiety disorders, was most substantially linked with the trait anxiety scale of the STAIC, whereas part two, which measures phobic symptoms, was most clearly associated with the FSSC-R-SF. The correlation between the YAM-5 and the SCAS was also robust, and particularly strong correlations were found between subscales of both questionnaires that assessed similar symptoms. Further, the selective mutism subscale of the YAM-5 was most clearly linked to the SMQ. Finally, the YAM-5 was also significantly correlated with depression symptoms as indexed by the CDI. These findings provide further support for the concurrent validity of the YAM-5. Copyright © 2017 Elsevier Ltd. All rights reserved.

Deep brain stimulation of the center median-parafascicular complex of the thalamus has efficient anti-parkinsonian action associated with widespread cellular responses in the basal ganglia network in a rat model of Parkinson's disease.

Science.gov (United States)

Jouve, Loréline; Salin, Pascal; Melon, Christophe; Kerkerian-Le Goff, Lydia

2010-07-21

The thalamic centromedian-parafascicular (CM/Pf) complex, mainly represented by Pf in rodents, is proposed as an interesting target for the neurosurgical treatment of movement disorders, including Parkinson's disease. In this study, we examined the functional impact of subchronic high-frequency stimulation (HFS) of Pf in the 6-hydroxydopamine-lesioned hemiparkinsonian rat model. Pf-HFS had significant anti-akinetic action, evidenced by alleviation of limb use asymmetry (cylinder test). Whereas this anti-akinetic action was moderate, Pf-HFS totally reversed lateralized neglect (corridor task), suggesting potent action on sensorimotor integration. At the cellular level, Pf-HFS partially reversed the dopamine denervation-induced increase in striatal preproenkephalin A mRNA levels, a marker of the neurons of the indirect pathway, without interfering with the markers of the direct pathway (preprotachykinin and preprodynorphin). Pf-HFS totally reversed the lesion-induced changes in the gene expression of cytochrome oxidase subunit I in the subthalamic nucleus, the globus pallidus, and the substantia nigra pars reticulata, and partially in the entopeduncular nucleus. Unlike HFS of the subthalamic nucleus, Pf-HFS did not induce per se dyskinesias and directly, although partially, alleviated L-3,4-dihydroxyphenylalanine (L-DOPA)-induced forelimb dyskinesia. Conversely, L-DOPA treatment negatively interfered with the anti-parkinsonian effect of Pf-HFS. Altogether, these data show that Pf-DBS, by recruiting a large basal ganglia circuitry, provides moderate to strong anti-parkinsonian benefits that might, however, be affected by L-DOPA. The widespread behavioral and cellular outcomes of Pf-HFS evidenced here demonstrate that CM/Pf is an important node for modulating the pathophysiological functioning of basal ganglia and related disorders.

Hopanoids play a role in stress tolerance and nutrient storage in the cyanobacterium Nostoc punctiforme.

Science.gov (United States)

Ricci, J N; Morton, R; Kulkarni, G; Summers, M L; Newman, D K

2017-01-01

Hopanes are abundant in ancient sedimentary rocks at discrete intervals in Earth history, yet interpreting their significance in the geologic record is complicated by our incomplete knowledge of what their progenitors, hopanoids, do in modern cells. To date, few studies have addressed the breadth of diversity of physiological functions of these lipids and whether those functions are conserved across the hopanoid-producing bacterial phyla. Here, we generated mutants in the filamentous cyanobacterium, Nostoc punctiforme, that are unable to make all hopanoids (shc) or 2-methylhopanoids (hpnP). While the absence of hopanoids impedes growth of vegetative cells at high temperature, the shc mutant grows faster at low temperature. This finding is consistent with hopanoids acting as membrane rigidifiers, a function shared by other hopanoid-producing phyla. Apart from impacting fitness under temperature stress, hopanoids are dispensable for vegetative cells under other stress conditions. However, hopanoids are required for stress tolerance in akinetes, a resting survival cell type. While 2-methylated hopanoids do not appear to contribute to any stress phenotype, total hopanoids and to a lesser extent 2-methylhopanoids were found to promote the formation of cyanophycin granules in akinetes. Finally, although hopanoids support symbiotic interactions between Alphaproteobacteria and plants, they do not appear to facilitate symbiosis between N. punctiforme and the hornwort Anthoceros punctatus. Collectively, these findings support interpreting hopanes as general environmental stress biomarkers. If hopanoid-mediated enhancement of nitrogen-rich storage products turns out to be a conserved phenomenon in other organisms, a better understanding of this relationship may help us parse the enrichment of 2-methylhopanes in the rock record during episodes of disrupted nutrient cycling. © 2016 John Wiley & Sons Ltd.

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

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Sourav Das

2015-12-01

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.

Organisk kataton tilstand efter apopleksi

DEFF Research Database (Denmark)

Jørgensen, Anders; Jørgensen, Martin Balslev

2009-01-01

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...

Diagnostic value of amplitude-phase analysis in myocardial infarct. Comparison with thallium perfusion scintigraphy and contrast ventrilography

International Nuclear Information System (INIS)

Garcheva, M.; Trindev, P.; Shejretova, E.; Stoyanova, N.; Kaloyanova, P.; Khadzhikostova, Kh.

1990-01-01

The evaluation is based on the results of investigation of 34 patients who have had myocardial infarct without rhythm disturbances. Compared to contrast ventrilography, the amplitude-phase analysis (APA) of 'rest' radionuclide ventrilography show 80% sensitivity and 100% specificity, as well as high accuracy in determination of the type and localization of the kinetic disturbances. The comparison with the thallium perfusion scintigraphy demonstrates the possibility of APA to vizualize abnormal kinetic area of the myocardial wall and shows its independent significance in the cases of doubtful findings. APA is a powerful tool for unambigious differentiating of hypokinetic from akinetic and diskinetic areas. 1 tab., 1 fig., 4 refs

Organisk kataton tilstand efter apopleksi

DEFF Research Database (Denmark)

Jørgensen, Anders; Jørgensen, Martin Balslev

2009-01-01

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-31...

Evaluation and management of the child with speech delay.

Science.gov (United States)

Leung, A K; Kao, C P

1999-06-01

A delay in speech development may be a symptom of many disorders, including mental retardation, hearing loss, an expressive language disorder, psychosocial deprivation, autism, elective mutism, receptive aphasia and cerebral palsy. Speech delay may be secondary to maturation delay or bilingualism. Being familiar with the factors to look for when taking the history and performing the physical examination allows physicians to make a prompt diagnosis. Timely detection and early intervention may mitigate the emotional, social and cognitive deficits of this disability and improve the outcome.

Comparision the value of detecting myocardial viability between low dose dobutamine stress MRI and echocardiography

International Nuclear Information System (INIS)

He Yi; Zhang Zhaoqi; Yu Wei; Miao Cuilian; Zhao Yike; Yan Zixu

2006-01-01

Objective: To Compare the diagnostic value between low dose dobutamine stress transthrotic echocardiography and low dose dobutamine stress MRI in detecting myocardial viability of chronic myocardial infarction. Methods: Rest and low dose dobutamine (5, 10 μg·kg -1 ·min -1 ) stress transthrotic echocardiography and cine-MRI were performed in 30 patients with chronic myocardial infarction. 24 patients underwent successful revascularization and 10 of them underwent another rest cine-MRI study to assess segmental myocardial functional recovery. Left ventricular were divided into 16 segments, the criteria of viability in different techniques is: MRI: dobutamine induced systolic wall thickening was ≥2 mm in akinetic or diskinetic segments at rest; Echocardiography: wall motion score reduced at least 1 after dobutamine stress in akinetic or' diskinetic segments at rest. Results: One hundren and eight segments showed wall motion abnormalities of 30 patients, 65 and 56 segments shows positive reaction, 43 and 52 segments shows negativereaction in MRI and echocardiography after dobutamine stress respectively. Kappa value of the two techniques is 0.75, concordance in both techniques is 88%. Twenty-four segments showed functional recovery, 14 segments remained dysfunction 3-6 months after revascularization, the sensitivity, specificity and accuracy of detecting myocardial viability in chronic myocardial infarction in MRI and echocardiography is 95.8% vs 79.2% (P>0.05), 85.7% vs 85.7% (P>0.05), 92.1% vs 81.6% (P>0.05) respectively. The sensitivity and accuracy of MRI is a little higher. Conclusion: The ability of detecting myocardial viability by both low dose dobutamine stress transthrotic echocardiography and low dose dobutamine stress MRI is similer, the sensitivity and accuracy of MRI is a little higher. (authors)

Screening for health: Tunisia

International Nuclear Information System (INIS)

2003-01-01

Imagine the distress for everyone involved when it becomes apparent that a child is mentally disabled, especially if the condition could have been avoided. Thyroid deficiency in newborn babies can lead to varying degrees of mental retardation or intellectual impairment and, in severe cases, can lead to deaf mutism. For the person affected and their family, life will never be normal and yet the condition can be treated effectively if it is detected within the first few days of life. Radioimmunoassay (RIA) is a simple, cost effective diagnostic technique. (IAEA)

Malignant Catatonia

Directory of Open Access Journals (Sweden)

Ayca Ozkul

2010-12-01

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.

Consensus paper on post-operative pediatric cerebellar mutism syndrome

DEFF Research Database (Denmark)

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

2016-01-01

-operative pediatric CMS" was formed, preliminary recommendations for diagnostic and follow-up procedures were created, two working groups on a new scoring scale and risk prediction and prevention were established, and areas were identified where further information is needed. DISCUSSION: The consensus process...... to provide a more solid foundation for future clinical and research work. It is thought as a consensus for moving forward and hopefully paves the way to developing a standard approach to this challenging problem with the advent of better scoring methods and ultimate goal of reducing the risk of CMS....

The Youth Anxiety Measure for DSM-5 (YAM-5): Development and First Psychometric Evidence of a New Scale for Assessing Anxiety Disorders Symptoms of Children and Adolescents.

Science.gov (United States)

Muris, Peter; Simon, Ellin; Lijphart, Hester; Bos, Arjan; Hale, William; Schmeitz, Kelly

2017-02-01

The Youth Anxiety Measure for DSM-5 (YAM-5) is a new self- and parent-report questionnaire to assess anxiety disorder symptoms in children and adolescents in terms of the contemporary classification system. International panels of childhood anxiety researchers and clinicians were used to construct a scale consisting of two parts: part one consists of 28 items and measures the major anxiety disorders including separation anxiety disorder, selective mutism, social anxiety disorder, panic disorder, and generalized anxiety disorder, whereas part two contains 22 items that focus on specific phobias and (given its overlap with situational phobias) agoraphobia. In general, the face validity of the new scale was good; most of its items were successfully linked to the intended anxiety disorders. Notable exceptions were the selective mutism items, which were frequently considered as symptoms of social anxiety disorder, and some specific phobia items especially of the natural environment, situational and other type, that were regularly assigned to an incorrect category. A preliminary investigation of the YAM-5 in non-clinical (N = 132) and clinically referred (N = 64) children and adolescents indicated that the measure was easy to complete by youngsters. In addition, support was found for the psychometric qualities of the measure: that is, the internal consistency was good for both parts, as well as for most of the subscales, the parent-child agreement appeared satisfactory, and there was also evidence for the validity of the scale. The YAM-5 holds promise as a tool for assessing anxiety disorder symptoms in children and adolescents.

The relationship between the First World War and neurology: 100 years of "Shell Shock".

Science.gov (United States)

Pedroso, José Luiz; Linden, Stefanie C; Barsottini, Orlando G; Maranhão, Péricles; Lees, Andrew J

2017-05-01

The First World War was a global war, beginning on 28 July 1914, until 11 November 1918. Soon after the beginning of the war, there was an "epidemic" of neurological conversion symptoms. Soldiers on both sides started to present in large numbers with neurological symptoms, such as dizziness, tremor, paraplegia, tinnitus, amnesia, weakness, headache and mutism of psychosomatic origin. This condition was known as shell shock, or "war neurosis". Because medically unexplained symptoms remain a major challenge, and considering the close relationship of symptoms described in shell shock with clinical neurology, we should study their history in order to improve future care.

Undiagnosed intraoperative methaemoglobinaemia

Directory of Open Access Journals (Sweden)

Swapnil Verma

2018-01-01

Full Text Available Methaemoglobinaemia is a rare but potentially dangerous haemoglobinopathy that is often underdiagnosed. It is one of the causes for unexplained cyanosis with dark-coloured blood, especially in the absence of cardiac or pulmonary pathology. Not uncommonly so, it is an incidental perioperative finding in cases of dark-coloured blood not improving with oxygen in apparently acyanotic patients. The present case report is of a child with deaf-mutism posted for cochlear implant surgery who presented with 'chocolate-coloured blood' in the surgical field, despite blood gas analysis showing a normal partial pressure of oxygen.

Lack of Methylated Hopanoids Renders the Cyanobacterium Nostoc punctiforme Sensitive to Osmotic and pH Stress.

Science.gov (United States)

Garby, Tamsyn J; Matys, Emily D; Ongley, Sarah E; Salih, Anya; Larkum, Anthony W D; Walter, Malcolm R; Summons, Roger E; Neilan, Brett A

2017-07-01

To investigate the function of 2-methylhopanoids in modern cyanobacteria, the hpnP gene coding for the radical S -adenosyl methionine (SAM) methylase protein that acts on the C-2 position of hopanoids was deleted from the filamentous cyanobacterium Nostoc punctiforme ATCC 29133S. The resulting Δ hpnP mutant lacked all 2-methylhopanoids but was found to produce much higher levels of two bacteriohopanepentol isomers than the wild type. Growth rates of the Δ hpnP mutant cultures were not significantly different from those of the wild type under standard growth conditions. Akinete formation was also not impeded by the absence of 2-methylhopanoids. The relative abundances of the different hopanoid structures in akinete-dominated cultures of the wild-type and Δ hpnP mutant strains were similar to those of vegetative cell-dominated cultures. However, the Δ hpnP mutant was found to have decreased growth rates under both pH and osmotic stress, confirming a role for 2-methylhopanoids in stress tolerance. Evidence of elevated photosystem II yield and NAD(P)H-dependent oxidoreductase activity in the Δ hpnP mutant under stress conditions, compared to the wild type, suggested that the absence of 2-methylhopanoids increases cellular metabolic rates under stress conditions. IMPORTANCE As the first group of organisms to develop oxygenic photosynthesis, Cyanobacteria are central to the evolutionary history of life on Earth and the subsequent oxygenation of the atmosphere. To investigate the origin of cyanobacteria and the emergence of oxygenic photosynthesis, geobiologists use biomarkers, the remnants of lipids produced by different organisms that are found in geologic sediments. 2-Methylhopanes have been considered indicative of cyanobacteria in some environmental settings, with the parent lipids 2-methylhopanoids being present in many contemporary cyanobacteria. We have created a Nostoc punctiforme Δ hpnP mutant strain that does not produce 2-methylhopanoids to assess the

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

Science.gov (United States)

Kamble, N; Netravathi, M; Saini, J; Mahadevan, A; Yadav, R; Nalini, A; Pal, P K; Satishchandra, P

2015-01-01

Autoimmune neuronal synaptic encephalitis (AIE) encompasses a heterogeneous group of disorders characterized by immune-mediated neuronal cell destruction. In this study, we aim to study the clinical features, imaging profile and treatment outcome of patients with AIE. This is a chart review of 16 (M: F-3:13) patients with AIE from 2011 to 2015. Among the patients, 10 had anti-NMDA, 4 had anti-TPO, and 2 had anti-VGKC antibody positivity. Cognitive impairment and seizures were the predominant symptoms present in nearly all patients, followed by psychiatric disturbances (87.5%), mutism (62.5%), movement disorders (62.5%), myoclonic jerks (37.5%) and visual hallucinations (18.75%). Magnetic resonance imaging (MRI) of the brain was available in 15 patients; it was abnormal in 53.3% patients. Abnormalities were seen in all patients with anti-VGKC positivity; and, 60% of patients with anti-NMDA positivity. Imaging was normal in 26.7% of the patients (3: anti-NMDA; and, 1: anti-TPO positivity); and, diffuse cerebral atrophy was noted in rest of the 20% (3: anti-TPO positivity) patients. All patients improved gradually with immunomodulation. 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.

Frontal Tasks and Behavior in Rigid or Tremor-Dominant Parkinson Disease.

Science.gov (United States)

Moretti, Rita; Milner, Vera; Caruso, Paola; Gazzin, Silvia; Rumiati, Raffaella

2017-08-01

Parkinson disease (PD) is not an unambiguous entity, and there is a general consensus for the statement that an akinetic-rigid dominant type of presentation has a worse prognosis, in the follow-up. The aim of our study was to examine the differences in frontal tasks and behavior, in 2 PD naive groups: the rigid and the tremor-dominant types of presentation, according to motor scores. Our study has showed some important differences in frontal tasks and in behavior, performing more apathy, aggressiveness, and irritability in the rigid type, and more depression and anxiety in the tremor-dominant type. The former group causes the caregiver more distress and has a very rapid disease progression. It can be argued that rigid type PD presentation needs specific dedicated cares and more strong clinical attention.

Striatal FP-CIT uptake differs in the subtypes of early Parkinson's disease

International Nuclear Information System (INIS)

Spiegel, J.; Fassbender, K.; Dillmann, U.; Hellwig, D.; Samnick, S.; Moellers, M.-O.; Kirsch, C.-M.; Jost, W.

2007-01-01

In idiopathic Parkinson's disease (PD), a tremor-dominant type (TDT), an akinetic-rigid type (ART), and a mixed type (MT) are distinguished. We compared cerebral [I- 123 ]FP-CIT SPECT in the PD subtypes (67 patients Hoehn and Yahr stage 1:26 with ART, 19 with MT, 22 with TDT). We measured the ratios putamen/occipital lobe binding and caudate nucleus/occipital lobe binding. Parkinsonian motor symptoms were quantified by UPDRS motor scale. In both putamen and caudate nucleus contralateral to the clinically affected body side TDT patients showed a significantly higher FP-CIT uptake than ART or MT patients (ANOVA; p 0.05). The missing correlation between striatal FP-CIT uptake and tremor suggests, that further systems besides the nigrostriatal dopaminergic system may contribute to generation of parkinsonian tremor. (author)

Huntington disease: a case study of early onset presenting as depression.

Science.gov (United States)

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

2004-10-01

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 rigidity, is reported. Meanwhile, the father developed the adult variant of Huntington disease. The boy's diagnosis was confirmed by molecular genetic analysis and magnetic resonance imaging. It is important to be aware of hereditary conditions such as Huntington disease and to provide family counseling before genetic testing and after the diagnosis is confirmed.

[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)].

Science.gov (United States)

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

1976-08-01

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.

Sonographic diagnosis of intestinal obstruction in the dog.

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Manczur, F; Vörös, K; Vrabély, T; Wladár, S; Németh, T; Fenyves, B

1998-01-01

Ultrasonography was performed on 44 dogs to decide whether small bowel obstruction was present. The sonographic criteria for small bowel obstruction were (1) the presence of pendulous movement of the ingesta inside the dilated bowel, (2) observation of invaginated intestines or an ingested intraluminal foreign body, (3) observation of non-uniform peristaltic activity of the dilated intestines, or (4) observation of akinetic intestinal loops together with abdominal fluid accumulation. By using these criteria, obstruction was correctly diagnosed by ultrasonography in 11 of the 13 dogs with mechanical ileus, and obstruction was correctly excluded in 29 of the 31 non-obstructive cases. Thus, the above-mentioned sonographic criteria had 85% sensitivity and positive predictive value, and 94% specificity and negative predictive value. The present study suggests that ultrasonography is a valuable tool for diagnosing small intestinal obstruction in the dog.

Wave Telescope Technique for MMS Magnetometer

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Narita, Y.; Plaschke, F.; Nakamura, R.; Baumjojann, W.; Magnes, W.; Fischer, D.; Voros, Z.; Torbert, R. B.; Russell, C. T.; Strangeway, R. J.;

[Functional pathophysiology of consciousness].

Science.gov (United States)

Jellinger, Kurt A

2009-01-01

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.

Catatonic syndrome associated with lead intoxication: a case report.

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Modabbernia, Mohammad Jafar; Mirsafa, Ali Reza; Modabbernia, Amirhossein; Pilehroodi, Farhad; Shirazi, Maryam

2009-08-11

Little is known about catatonia associated with lead intoxication. A retired printing house worker man presented with one week history of refusal to eat and mutism. He was treated with possible diagnosis of catatonia with administration of Lorazepam 3 mg P.O. daily. Significant improvement occurred after 48 hours. In further examinations, there was no evidence of physical and mental disorders while impairment in neuropsychiatry test, identification of Dohle body, basophilic stippling and toxic granulation in peripheral blood smear and blood lead level of 12.8 mug/dl were recorded. Possibly, lead intoxication results in changes in neurotransmitter system that leads to catatonia. Lorazepam improves patient's condition through changes in this system.

[Inpatient treatment of a child with elective mutism--a case report].

Science.gov (United States)

Wernitznig, H

1993-01-01

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.

Left ventricular pseudoaneurysm versus aneurysm a diagnosis dilemma

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Samarjit Bisoyi

2016-01-01

Full Text Available Free wall rupture of the left ventricle (LV is a rare but life-threatening complication of acute myocardial infaction. Very rarely such rupture may be contained by the adhering pericardium creating a pseudoaneurysm. This condition warrants for an emergency surgery. Left ventricular aneurysm is the discrete thinning of the ventricular wall (<5 mm with akinetic or dyskinetic wall motion causing an out-pouching of the ventricle. Given the propensity for pseudoaneurysms to rupture leading to cardiac tamponade, shock, and death, compared with a more benign natural history for true aneurysms, accurate diagnosis of these conditions is important. True aneurysm, usually, calls for an elective surgery. Clinically differentiating the two conditions remains a challenge. We report the case of a patient with LV pseudoaneurysm, initially diagnosed as true aneurysm at our institution. We have attempted to review the existing literature and discussed the characteristic findings of each entity.

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

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Marcelo Pinto Pereira

2013-09-01

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.

Serotonin syndrome associated with sertraline use: case report

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Bárbara Werner Griciunas

2017-06-01

Full Text Available Case report of serotonin syndrome in patient who initiated the use of sertraline at a dose greater than twice the recommended for the treatment of psychotic depression. The patient presented contracture of the limbs, puzzled look, mutism and blood pressure 230x110 mmHg. The syndrome is increasingly common, although it is not well recognized. Many medications can cause it and this possibility should be considered in patients taking serotonergic drugs presenting autonomic or mental disorders and neurological symptoms. The findings of clonus, oculogyric crisis, hyperreflexia and hypertonicity should lead to the medication review. Treatment focuses on interruption of causative agents, treatment of a possible hyperthermia and use of benzodiazepines to decrease hypertonus and neurological excitability.

[Case with difficulty in differentiating between transient neuroleptic malignant syndrome and catatonia after neuroleptic analgesia].

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Yanagawa, Youichi; Miyazaki, Masaki

2010-02-01

An 18-year-old woman was treated with neuroleptic analgesia using fentanyl, morphine, droperidol and haloperidol for general anesthesia and pain control for her knee operation. Postoperatively, she showed emotional unstableness, following dyspnea, tachycardia, fever, hyperhydrosis, muscle rigidity and myoclonus like involuntary movement. She received infusion of 140 mg dantrolene in total under suspicion of having neuroleptic malignant syndrome, but her symptoms improved slightly. After being transferred to our hospital, she exhibited immobility, mutism, rigidity, and catalepsy, and she was suspected of having lethal catatonia. Infusion of diazepam 10 mg resulted in dramatical improvement of her symptoms. Differential diagnosis between neuroleptic malignant syndrome and catatonia is difficult; however, a first line therapy is differential diagnosis. Thus, physician should consider catatonia when treating neuroleptic malignant like syndrome.

Renal Failure in Dementia with Lewy Bodies Presenting as Catatonia

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Fekete, Robert

2013-01-01

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. PMID:23466522

Anti-Ma2-associated encephalitis with normal FDG-PET: a case of pseudo-Whipple's disease.

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Castle, James; Sakonju, Ai; Dalmau, Josep; Newman-Toker, David E

2006-10-01

A 39-year-old man presented with a history of several months of progressive personality changes, social withdrawal, bradykinesia, mutism, dysphagia, worsening gait, and difficulty with daily living activities. Examination revealed an atypical parkinsonian appearance with incomplete supranuclear ophthalmoplegia and an unusual oculomotor disorder characterized by both low-amplitude, intermittent opsoclonus, and slow, nystagmoid intrusions. Routine laboratory testing, autoimmune and infectious serologies, brain MRI, lumbar puncture, electroencephalogram, whole-body CT scan, paraneoplastic serologies, small bowel biopsy, 18F-fluorodeoxyglucose positron emission tomography CT scan, brain biopsy, and testicular ultrasound. Anti-Ma2 paraneoplastic encephalitis in association with metastatic testicular cancer; initially misdiagnosed as CNS Whipple's disease. Corticosteroids, intravenous immunoglobulins, orchiectomy, muscle relaxants, mycophenolate mofetil, plasmapheresis, and bleomycin, etoposide and platinum chemotherapy.

Evaluation of regional wall motion abnormalities of the heart. Comparison with Doppler tissue echocardiography, MR-tagging and levocardiography

International Nuclear Information System (INIS)

Kivelitz, D.E.; Enzweiler, C.N.H.; Hamm, B.; Borges, A.C.; Walde, T.; Rutsch, W.; Baumann, G.

2004-01-01

Purpose: To compare the visual analysis of magnetic resonance imaging (MRI) with the tagging technique and Doppler tissue echocardiography with invasive ventriculography in detecting and quantifying regional left ventricular wall motion abnormalities. Materials and Methods: Sixteen patients with coronary artery disease and a history of prior myocardial infarction underwent invasive ventriculography. Doppler tissue echocardiography and MR-tagging within one week. Regional wall motion abnormalities (WMA) were detected in all patients. WMA were graded as normal=1; hypokinetic=2; akinetic=3; or dyskinetic=4. For agreement between MRI, echocardiography, and ventriculography the kappa coefficient (κ) according to Cohen was calculated. Results: The kappa coefficient (κ) was 0.962 for agreement between MRI and echocardiography and 0.602 for agreement between MRI and ventriculography as well as between echocardiography and ventriculography. Conclusion: Reliable analysis of regional left ventricular wall motion abnormalities is feasible using visual analysis of MR-tagging. MRI and Doppler tissue echocardiography detect more WMA than invasive ventriculography and grade them as more severe. (orig.)

The relationship between myocardial blood flow and myocardial viability after reperfusion. Myocardial viability assessed by 15O-water-PET

International Nuclear Information System (INIS)

Tsukagoshi, Joichi

1994-01-01

The purpose of this study was to examine the relationship between myocardial blood flow and myocardial viability in the ischemic canine myocardium after reperfusion. Transient ischemia was induced by 60-, 90-, and 180-minute occlusion of the left anterior descending coronary artery. Myocardial blood flow (MBF) was measured in the areas in which regional contractility was severely impaired (ehocardiographically akinetic or dyskinetic) in the early reperfusion period by 15 O-water positron emission tomography (PET) 12 hours and 4 weeks after reperfusion. An MBF ratio of ischemic to nonischemic regions 12 hours after reperfusion was inversely correlated with the amount of histologically determined tissue necrosis (r=-0.74). The regional contractility recovered 4 weeks later in the areas where an MBF ratio was 0.48 or greater, but did not recover in the areas with a lower MBF ratio. Thus, myocardial viability can be appropriately predicted in the early phase of myocardial perfusion by PET with 15 O-water even in the absence of metabolic imaging. (author)

Regional ejection fraction: a quantitative radionuclide index of regional left ventricular performance

International Nuclear Information System (INIS)

Maddox, D.E.; Wynne, J.; Uren, R.; Parker, J.A.; Idoine, J.; Siegel, L.C.; Neill, J.M.; Cohn, P.F.; Holman, B.L.

1979-01-01

Left ventricular regional ejection fractions were derived from background-corrected, time-activity curves in 43 patients assessed by both gated equilibrium radionuclide angiocardiography and left ventricular contrast angiography. From a single, modified left anterior oblique projection, the regional change in background corrected counts was determined in each of three anatomic regions. The normal range for regional radionuclide ejection fraction was determined in 10 patients with normal contrast ventriculograms and without obstructive coronary artery disease at coronary arteriography. Regional ejection fraction was compared with percent segmental axis shortening and extent of akinetic segments in corresponding regions of the contrast ventriculogram. Radionuclide and roentgenographic methods were in agreement as to the presence or absence of abnormal wall motion in 83 of 99 left ventricular regions (84%) in 33 patients evaluated prospectively. Comparison of regional ejection fraction demonstrated significant differences between regions with roentgenographically determined normokinesis hypokinesis, and akinesis. We conclude that the left ventricular regional ejection fraction provides a reliable quantitative assessment of regional left ventricular performance

Cellular responses in the cyanobacterial symbiont during its vertical transfer between plant generations in the Azolla microphylla symbiosis.

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Zheng, Weiwen; Bergman, Birgitta; Chen, Bin; Zheng, Siping; Guan, Xiong; Xiang, Guan; Rasmussen, Ulla

2009-01-01

The nitrogen-fixing symbiosis between cyanobacteria and the water fern Azolla microphylla is, in contrast to other cyanobacteria-plant symbioses, the only one of a perpetual nature. The cyanobacterium is vertically transmitted between the plant generations, via vegetative fragmentation of the host or sexually within megasporocarps. In the latter process, subsets of the cyanobacterial population living endophytically in the Azolla leaves function as inocula for the new plant generations. Using electron microscopy and immunogold-labeling, the fate of the cyanobacterium during colonization and development of the megasporocarp was revealed. On entering the indusium chamber of the megasporocarps as small-celled motile cyanobacterial filaments (hormogonia), these differentiated into large thick-walled akinetes (spores) in a synchronized manner. This process was accompanied by cytoplasmic reorganizations and the release of numerous membrane vesicles, most of which contained DNA, and the formation of a highly structured biofilm. Taken together the data revealed complex adaptations in the cyanobacterium during its transition between plant generations.

Evaluation of segmental left ventricular wall motion by equilibrium gated radionuclide ventriculography.

Science.gov (United States)

Van Nostrand, D; Janowitz, W R; Holmes, D R; Cohen, H A

1979-01-01

The ability of equilibrium gated radionuclide ventriculography to detect segmental left ventricular (LV) wall motion abnormalities was determined in 26 patients undergoing cardiac catheterization. Multiple gated studies obtained in 30 degrees right anterior oblique and 45 degrees left anterior oblique projections, played back in a movie format, were compared to the corresponding LV ventriculograms. The LV wall in the two projections was divided into eight segments. Each segment was graded as normal, hypokinetic, akinetic, dyskinetic, or indeterminate. Thirteen percent of the segments in the gated images were indeterminate; 24 out of 27 of these were proximal or distal inferior wall segments. There was exact agreement in 86% of the remaining segments. The sensitivity of the radionuclide technique for detecting normal versus any abnormal wall motion was 71%, with a specificity of 99%. Equilibrium gated ventriculography is an excellent noninvasive technique for evaluating segmental LV wall motion. It is least reliable in assessing the proximal inferior wall and interventricular septum.

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

Energy Technology Data Exchange (ETDEWEB)

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

1986-01-13

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.

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

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Yvette M. Bordelon

2011-01-01

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.

[Anxiety disorders in DSM-5].

Science.gov (United States)

Márquez, Miguel

2014-01-01

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.

Renal Failure in Dementia with Lewy Bodies Presenting as Catatonia

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Robert Fekete

2013-01-01

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.

A case with basilar artery thrombosis resulted in Locked-in syndrome in spite of endovascular treatment

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Yusuf İnanç

2015-04-01

Full Text Available Locked-in Sendrome is a clinical picture consist of quadriplegia, lower cranial nerve paralysis, and mutism with preservation of only vertical gaze and upper eyelid movement. Consciousness remains intact and the patient is able to communicate intentionally using eye blinking. The most common cause underlying the locked-in syndrome is thrombosis of the basilar artery. In this study, we reported a 49-years-old male with past medical history for cerebrovascular disease presented with acute basilar artery thrombosis, manifesting as reduced level of consciousness, weakness in all extremity dominated on the right side, speech impairment, horizontal gaze disorder and for reaching us of the last munite of endovascular intervention threshold, so it can perform only mechanical and intra-arterial thrombosis treatment method as an endovascular treatment modalities of acute stroke.

Cerebellar mutism syndrome in children with brain tumours of the posterior fossa

DEFF Research Database (Denmark)

Wibroe, Morten; Cappelen, Johan; Castor, Charlotte

2017-01-01

, the clinical course and strategies for prevention and treatment are yet to be determined.Methods: This observational, prospective, multicentre study will include 500 children with posterior fossa tumours. It opened late 2014 with participation from 20 Nordic and Baltic centres. From 2016, five British centres...... for and the clinical course of CMS - with the ultimate goal of defining strategies for prevention and treatment of this severely disabling condition....

Cerebellar mutism syndrome in children with brain tumours of the posterior fossa

DEFF Research Database (Denmark)

Wibroe, Morten; Cappelen, Johan; Castor, Charlotte

2017-01-01

and four Dutch centres will join with a total annual accrual of 130 patients. Three other major European centres are invited to join from 2016/17. Follow-up will run for 12 months after inclusion of the last patient. All patients are treated according to local practice. Clinical data are collected through...

Restoration of optimal ellipsoid left ventricular geometry: lessons learnt from in silico surgical modelling.

Science.gov (United States)

Adhyapak, Srilakshmi M; Menon, Prahlad G; Rao Parachuri, V

2014-02-01

Several issues that are inherent in the surgical techniques of surgical ventricular restoration (SVR) need specialized devices or techniques to overcome them, which may not always result in optimal outcomes. We used a non-invasive novel in silico modelling technique to study left ventricular (LV) morphology and function before and after SVR. The cardiac magnetic resonance imaging derived actual pre- and postoperative endocardial morphology and function was compared with the in silico analysis of the same. Cardiac magnetic resonance steady state free precession (SSFP) cine images were employed to segment endocardial surface contours over the cardiac cycle. Using the principle of Hausdorff distance to examine phase-to-phase regional endocardial displacement, dyskinetic/akinetic areas were identified at the instant of peak basal contraction velocity. Using a three-dimensional (3D) surface clipping tool, the maximally scarred, dyskinetic or akinetic LV antero-apical areas were virtually resected and a new apex was created. A virtual rectangular patch was created upon the clipped surface LV model by 3D Delaunay triangulation. Presurgical endocardial mechanical function quantified from cine cardiac magnetic resonance, using a technique of spherical harmonics (SPHARM) surface parameterization, was applied onto the virtually clipped and patched LV surface model. Finally, the in silico model of post-SVR LV shape was analysed for quantification of regional left ventricular volumes (RLVVs) and function. This was tested in 2 patients with post-myocardial infarction antero-apical LV aneuryms. Left ventricular mechanical dysynchrony was evaluated by RLVV analysis of pre-SVR, in silico post-SVR and actual post-SVR LV endocardial surface data. Following exclusion of the scarred areas, the virtual resected LV model demonstrated significantly lesser areas of akinesia. The decreases in regional LV volumes in the in silico modelling were significant and comparable with the actual

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

2007-06-01

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

[An improved case of bedridden mental impairment with normal pressure hydrocephalus associated with acoustic neurinoma after tumor resection].

Science.gov (United States)

Sugimoto, Seiichiro; Sugimoto, Akiko; Saita, Kazuko; Kishi, Masahiko; Shioya, Keiichi; Higa, Toshinobu

2008-08-01

A 67-year-old woman developed gait disturbance, dysarthria, cognitive impairment and incontinence at age 65, and became bedridden. She showed mutism, stupor and lower limb spasticity. Cranial CT and MRI revealed marked ventricular enlargement and a cerebellopontine angle tumor. CSF study showed normal pressure (125 mmH2O) and elevated protein (143 mg/dl). Radionuclide cisternography showed redistribution of radionuclide to the ventricles and intraventricular residual radionuclide after 72 hours, which allowed a diagnosis of normal pressure hydrocephalus. After removal of the tumor, ventricle size and CSF protein decreased, and the symptoms of cognitive impairment and motor dysfunction resolved. Histological examination showed acoustic neurinoma. Over the half of hydrocephalus following acoustic neurinoma shows a tendency to improve by surgical resection of the tumor. Neurologists who see cognitively impaired spastic bedridden patients should not overlook this pathology.

Japanese encephalitis in a French traveler to Nepal.

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Lagarde, S; Lagier, J-C; Charrel, R; Quérat, G; Vanhomwegen, J; Desprès, P; Pelletier, J; Kaphan, E

2014-02-01

Japanese encephalitis is frequent in Asia, with a severe prognosis, but rare in travelers. Culex mosquitoes transmit Japanese encephalitis virus. Risk factors are destination, duration of stay, summer and fall seasons, outdoor activities, and type of accommodation. We report the case of a French traveler to Nepal with neutralization-based serological confirmed Japanese encephalitis. He presented classical clinical (viral syndrome before an encephalitis status with behavioral disorder, global hypotonia, mutism, movement disorders, seizure, and coma), radiological (lesions of thalami, cortico-spinal tracts, and brainstem) and biological features (lymphocytic meningitis). Nowadays, the presence of Japanese encephalitis virus in Nepal, including mountain areas, is established but Japanese encephalitis remains rare in travelers returning from this area and neurologist physicians need to become familiar with this. We recommend vaccination for travelers spending a long period of time in Nepal and having at-risk outdoor activities.

Evaluation of viability of infarcted myocardium by low dose dobutamine stress echocardiography. Comparison with exercise stress 201Tl myocardial scintigraphy

International Nuclear Information System (INIS)

Nagahara, Toshihiro; Sakamoto, Kazunori; Sofue, Akira; Horiuchi, Toshimitsu; Yamazaki, Shigeki; Kuwako, Kenji

1999-01-01

Exercise stress 201 Tl myocardial single-photon emission computed tomography (SPECT) is recognized to be a excellent method for identifying viability after myocardial infarction, but it is expensive and needs a longer time for data acquisition than echocardiography. We therefore performed this study to evaluate the effectiveness of low dose (5-10 μg/kg/min) dobutamine stress echocardiography (DSE) in 30 patients (61±8 years old: 24 men and 6 women) within 4 weeks after myocardial infarction in identifying viable myocardium, compared to results obtained by SPECT. Defining an akinetic or dyskinetic segment obtained by rest echocardiography as a definite infarct area, altogether 96 segments out of 716 segments were shown to be infarct areas. Of these, 75 (78%) segments were identified as viable by DSE, and 77 (80%) by SPECT. Only 2 segments were shown to be discrepant on DSE and SPECT. Subsequently, the sensitivity, specificity and accuracy rates for DSE were 96%, 100% and 96%, respectively. In conclusion, DSE is as effective and useful as SPECT in the evaluation of viability after myocardial infarction. (author)

The relationship between myocardial blood flow and myocardial viability after reperfusion. Myocardial viability assessed by [sup 15]O-water-PET

Energy Technology Data Exchange (ETDEWEB)

Tsukagoshi, Joichi (Gunma Univ., Maebashi (Japan). School of Medicine)

1994-09-01

The purpose of this study was to examine the relationship between myocardial blood flow and myocardial viability in the ischemic canine myocardium after reperfusion. Transient ischemia was induced by 60-, 90-, and 180-minute occlusion of the left anterior descending coronary artery. Myocardial blood flow (MBF) was measured in the areas in which regional contractility was severely impaired (ehocardiographically akinetic or dyskinetic) in the early reperfusion period by [sup 15]O-water positron emission tomography (PET) 12 hours and 4 weeks after reperfusion. An MBF ratio of ischemic to nonischemic regions 12 hours after reperfusion was inversely correlated with the amount of histologically determined tissue necrosis (r=-0.74). The regional contractility recovered 4 weeks later in the areas where an MBF ratio was 0.48 or greater, but did not recover in the areas with a lower MBF ratio. Thus, myocardial viability can be appropriately predicted in the early phase of myocardial perfusion by PET with [sup 15]O-water even in the absence of metabolic imaging. (author).

Rating scale for psychogenic nonepileptic seizures: scale development and clinimetric testing.

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Cianci, Vittoria; Ferlazzo, Edoardo; Condino, Francesca; Mauvais, Hélène Somma; Farnarier, Guy; Labate, Angelo; Latella, Maria Adele; Gasparini, Sara; Branca, Damiano; Pucci, Franco; Vazzana, Francesco; Gambardella, Antonio; Aguglia, Umberto

2011-06-01

Our aim was to develop a clinimetric scale evaluating motor phenomena, associated features, and severity of psychogenic nonepileptic seizures (PNES). Sixty video/EEG-recorded PNES induced by suggestion maneuvers were evaluated. We examined the relationship between results from this scale and results from the Clinical Global Impression (CGI) scale to validate this technique. Interrater reliabilities of the PNES scale for three raters were analyzed using the AC1 statistic, Kendall's coefficient of concordance (KCC), and intraclass correlation coefficients (ICCs). The relationship between the CGI and PNES scales was evaluated with Spearman correlations. The AC1 statistic demonstrated good interrater reliability for each phenomenon analyzed (tremor/oscillation, tonic; clonic/jerking, hypermotor/agitation, atonic/akinetic, automatisms, associated features). KCC and the ICC showed moderate interrater agreement for phenomenology, associated phenomena, and total PNES scores. Spearman's correlation of mean CGI score with mean total PNES score was 0.69 (Pscale described here accurately evaluates the phenomenology of PNES and could be used to assess and compare subgroups of patients with PNES. Copyright © 2011 Elsevier Inc. All rights reserved.

Value of 201-thallium serial myocardial imaging in coronary heart disease

International Nuclear Information System (INIS)

Sauer, E.; Sebening, H.; Dressler, J.; Lutilsky, L.; Ulm, K.; Hoer, G.; Papst, H.W.; Bloemer, H.; Technische Univ. Muenchen; Technische Univ. Muenchen

1979-01-01

There has been clinical evidence that a perfusion defect on a stress image fills overtime. The diagnostic value of initial and 120 min post exercise redistribution thallium-201 myocardial images (RMI) was determined in 120 pts, with suspected coronary heart disease (CAD), all of whom had coronary arteriography. Significant (>= 75%) lesions were present in 88 pts. 30 pts. without CAD showed a normal tracer uptake immediately after exercise. Scintigrams taken 120 min after exercise revealed a decrease of 201-Tl concentration in every area of the myocardium. 80 pts. with CAD showed an area of decreased tracer uptake in the inital scans. 120 min RMI in 51 pts. revealed a significant increase (p > 0.01) of countrate time ratio in previous underperfused areas. In 37 pts. persistent defects were present, in every case the defect correlated with the site of a myocardial infarction as determined by the finding of an akinetic area in the left ventricular angiogram. Thus RMI following a single dose of 201 Tl can differentiate between scar- and exercise-induced transient ischemia. (orig.) 891 AJ/orig. 892 MB [de

Brugada syndrome and calcium channel mutation in a patient with congenital deaf mutism

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Uğur Canpolat

2017-01-01

Full Text Available To the best of our knowledge, for the first time in the literature, we described a congenitally deaf-mute patient with Brugada syndrome (BrS in whom a mutation in L-type Ca+2 channel [CACNA1C (Cav1.2α1] was identified.

Abnormal laughter-like vocalisations replacing speech in primary progressive aphasia

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Rohrer, Jonathan D.; Warren, Jason D.; Rossor, Martin N.

2009-01-01

We describe ten patients with a clinical diagnosis of primary progressive aphasia (PPA) (pathologically confirmed in three cases) who developed abnormal laughter-like vocalisations in the context of progressive speech output impairment leading to mutism. Failure of speech output was accompanied by increasing frequency of the abnormal vocalisations until ultimately they constituted the patient's only extended utterance. The laughter-like vocalisations did not show contextual sensitivity but occurred as an automatic vocal output that replaced speech. Acoustic analysis of the vocalisations in two patients revealed abnormal motor features including variable note duration and inter-note interval, loss of temporal symmetry of laugh notes and loss of the normal decrescendo. Abnormal laughter-like vocalisations may be a hallmark of a subgroup in the PPA spectrum with impaired control and production of nonverbal vocal behaviour due to disruption of fronto-temporal networks mediating vocalisation. PMID:19435636

A novel de novo mutation in ATP1A3 and childhood-onset schizophrenia

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Smedemark-Margulies, Niklas; Brownstein, Catherine A.; Vargas, Sigella; Tembulkar, Sahil K.; Towne, Meghan C.; Shi, Jiahai; Gonzalez-Cuevas, Elisa; Liu, Kevin X.; Bilguvar, Kaya; Kleiman, Robin J.; Han, Min-Joon; Torres, Alcy; Berry, Gerard T.; Yu, Timothy W.; Beggs, Alan H.; Agrawal, Pankaj B.; Gonzalez-Heydrich, Joseph

2016-01-01

We describe a child with onset of command auditory hallucinations and behavioral regression at 6 yr of age in the context of longer standing selective mutism, aggression, and mild motor delays. His genetic evaluation included chromosomal microarray analysis and whole-exome sequencing. Sequencing revealed a previously unreported heterozygous de novo mutation c.385G>A in ATP1A3, predicted to result in a p.V129M amino acid change. This gene codes for a neuron-specific isoform of the catalytic α-subunit of the ATP-dependent transmembrane sodium–potassium pump. Heterozygous mutations in this gene have been reported as causing both sporadic and inherited forms of alternating hemiplegia of childhood and rapid-onset dystonia parkinsonism. We discuss the literature on phenotypes associated with known variants in ATP1A3, examine past functional studies of the role of ATP1A3 in neuronal function, and describe a novel clinical presentation associated with mutation of this gene. PMID:27626066

Maintenance electroconvulsive therapy for depression with catatonia in a young woman with Down syndrome.

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Torr, Jennifer; D'Abrera, Juan Carlos

2014-12-01

To describe and discuss the use of maintenance electroconvulsive therapy (ECT) in a young woman with Down syndrome and depression with catatonia. Clinical case report. A 23-year-old woman with Down syndrome (mosaic type) and a 4-year history of depressed mood triggered by adverse life events presented with mutism, psychomotor retardation, and compromised oral intake. Multiple trials of antidepressant medications were either ineffective or complicated by adverse reactions. She improved rapidly with a course of bilateral ECT but required maintenance ECT to sustain recovery. A series of premorbid, morbid, and post-treatment drawings by the young woman highlight the efficacy of treatment. Electroconvulsive therapy was found to be a safe and effective treatment for life-threatening mental illness in a young woman with Down syndrome who had failed multiple trials of antidepressant medications. This case highlights the importance of considering catatonia as a diagnosis in persons with Down syndrome and the effectiveness of electroconvulsive treatment.

Electroconvulsive therapy in a patient with moyamoya syndrome.

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Ghignone, Erica; Rosenthal, Lisa; Lloyd, Robert Brett; Mouli, Samdeep; Dinwiddie, Stephen

2015-03-01

We report on a 30-year-old woman diagnosed with moyamoya syndrome resulting from sickle cell disease who developed catatonia and was successfully treated with electroconvulsive therapy (ECT). Neuroimaging revealed severe tandem narrowing of the left internal carotid artery with diminished cerebral blood flow, moderate narrowing of the right supraclinoid aspect of the right internal carotid artery, and associated numerous lenticulostriate collaterals bilaterally, consistent with moyamoya. The patient presented with mutism; posturing; immobility; stupor; withdrawal; refusal to eat, drink, or speak; and staring, supporting a diagnosis of catatonia. It initially responded to a lorazepam challenge; however, a complicated hospital course and deterioration of the patient's condition, including septic shock, delirium, and continued catatonic symptoms, led to the pursuit of ECT to treat her symptoms. We discuss the risks involved with the administration of ECT in a patient with fragile cerebral vasculature and the successful treatment of catatonia in this patient without resultant stroke or cerebral hemorrhage.

Catatonia from its creation to DSM-V: Considerations for ICD.

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Fink, Max

2011-07-01

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.

IL BISOGNO DELL’ORIGINE E L’ORRORE DEL MUTISMO. ALCUNE NOTE SULLA QUESTIONE DELL’ORIGINE DEL LINGUAGGIO VERBALE UMANO MORE PHILOSOPHICO

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Salottolo Delio

2013-12-01

Full Text Available In this essay we analyze more philosophico the impact of the research on the origin of human verbal language. Starting from suggestions deriving from the work of Agamben and Lévi-Strauss, two discontinuists, we face a double contradiction. The first relates to the inability to define the birth date of human language and of human world. This consideration makes us suspect that behind the passion for this issue there is a hidden need. The second concerns the mix of fascinans and tremendum linked to human mutism, which suggests, from the dominator point of view, an attitude to human animalization, and, from the dominated point of view, a form of resistance or survival instinct. Under these lenses we will read the enfant sauvage by Itard and the Foe by Coetzee. Finally we will discuss the issue of the need for origin as modern mankind melancholia and as unresolved tension towards the future.

Magnetic resonance imaging of Parkinsonism

International Nuclear Information System (INIS)

Nakamura, Yusaku; Takahashi, Mitsuo; Kitaguchi, Masataka; Akaneya, Yukio; Mitui, Yoshiyuki; Tanaka, Hisashi

1991-01-01

We studied eighteen patients affected by Parkinsonism with symptoms of tremor, bradykinesia, or rigidity using magnetic resonance imaging (MRI). Patients ranged in age from 34 to 80 years (mean 62.8±11.6 years), and the duration of their disease had been 3.8±3.2 years. MRI examinations were performed with Shimazu and Siemens superconducting magnets, operating at 0.5 and 1.5 T magnetic fields, respectively. Both T 1 - and T 2 -weighted spin echo (SE) pulse sequences were used. In eight patients (44.4%), MRI demonstrated bilateral multiple lacunar infarction of the basal ganglia. The most common abnormality identified was multiple, bilateral lacunar infarcts in the lateral portion of the putamen. The average size of the lacunar infarction of the putamen was less than half that of the entire putamen. Patients with multiple lacunar infarction were significantly older than the other patients and had lower Yahr's scores. The clinical symptoms of patients with bilateral multiple lacunar infarction of the basal ganglia were compatible with the diagnosis of arteriosclerotic Parkinsonism of akinetic rigid type. It has been suggested that multiple lacunar infarction of the basal ganglia may have led to Parkinsonism in these patients. (author)

Voluptuary habits and clinical subtypes of Parkinson's disease: the FRAGAMP case-control study.

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Nicoletti, Alessandra; Pugliese, Pierfrancesco; Nicoletti, Giuseppe; Arabia, Gennarina; Annesi, Grazia; Mari, Michele De; Lamberti, Paolo; Grasso, Lucia; Marconi, Roberto; Epifanio, Antonio; Morgante, Letterio; Cozzolino, Autilia; Barone, Paolo; Torchia, Giusi; Quattrone, Aldo; Zappia, Mario

2010-10-30

We evaluated the possible association between smoking, coffee drinking, and alcohol consumption and Parkinson's disease (PD). The FRAGAMP study is a large Italian multicenter case-control study carried out to evaluate the possible role of environmental and genetic factors in PD. Adjusted ORs were estimated using unconditional logistic regression. Smoking, coffee, and alcohol consumption were also considered as surrogate markers of lifestyle and analysis was carried out considering the presence of at least one, two, or three factors. This latter analysis was separately performed considering Tremor-Dominant (TD) and Akinetic-Rigid (AR) patients. Four hundred ninety-two PD patients (292 men and 200 women) and 459 controls (160 men and 299 women) were enrolled in the study. Multivariate analysis showed a significant negative association between PD and cigarette smoking (OR 0.51; 95%CI 0.36-0.72), coffee drinking (OR 0.61; 95%CI 0.43-0.87) and wine consumption (OR 0.62; 95%CI 0.44-0.86); a significant trend dose-effect (P coffee, and alcohol consumption. When analysis was carried out considering the association of these factors as possible surrogate markers of a peculiar lifestyle the association was stronger for the AR phenotype.

Determination of left ventricular wall motility injury by factor analysis in patients with advanced ischemic heart disease

International Nuclear Information System (INIS)

Kasalicky, J.; Kidery, J.; Vavrejn, B.; Surova, H.; Malek, I.

1989-01-01

Left ventricular phase and amplitude images (Fourier analysis, PAI) and factor analysis images (FAI) from gated radionuclide ventriculography were obtained in 235 patients after myocardial infarction (MI) and in 44 patients with well documented ischemic heart disease (IHD) in order to assess areas of regional left ventricular motility injury (LVMI). The sensitivity of FAI for LVMI detection was higher than with PAI (36.3% vs 22.7% in patients without MI; 76.6% vs 68% in those after anterior MI; and 53.2% vs 31.9% after posterior MI, respectively). In 2.9% of all patients PAI were unclear due to small time activity amplitudes and heart rate irregularity, whereas FAI could be easily assessed. Significantly decreased left ventricular ejection fraction was observed predominantly after anterior MI in connection with distinct signs of LVMI in a large area of anterior wall or in the anteroseptal and/or apical region. Areas of LVMI could be sharply delineated in FAI; however, in contrast to PAI, FAI is unable to distinguish between dyskinetic and akinetic regions. The use of both PAI and FAI is recommended for more detailed detection of regional LVMI in patients with IHD. (orig.)

Do basal Ganglia amplify willed action by stochastic resonance? A model.

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V Srinivasa Chakravarthy

Full Text Available Basal ganglia are usually attributed a role in facilitating willed action, which is found to be impaired in Parkinson's disease, a pathology of basal ganglia. We hypothesize that basal ganglia possess the machinery to amplify will signals, presumably weak, by stochastic resonance. Recently we proposed a computational model of Parkinsonian reaching, in which the contributions from basal ganglia aid the motor cortex in learning to reach. The model was cast in reinforcement learning framework. We now show that the above basal ganglia computational model has all the ingredients of stochastic resonance process. In the proposed computational model, we consider the problem of moving an arm from a rest position to a target position: the two positions correspond to two extrema of the value function. A single kick (a half-wave of sinusoid, of sufficiently low amplitude given to the system in resting position, succeeds in taking the system to the target position, with high probability, only at a critical noise level. But for suboptimal noise levels, the model arm's movements resemble Parkinsonian movement symptoms like akinetic rigidity (low noise and dyskinesias (high noise.

Picrotoxin-induced behavioral tolerance and altered susceptibility to seizures: effects of naloxone.

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Thomas, J; Nores, W L; Pariser, R

1993-07-01

The role of opiate mechanisms in the development of tolerance and altered susceptibility to seizures after repeated injections of picrotoxin was investigated. Independent groups of rats were pretreated with naloxone (0.3, 1.0, 3.0, and 10.0 mg/kg) or the saline vehicle and then tested for seizures induced by picrotoxin. The procedure was performed on 3 days at 1-week intervals, for a total of 3 testing days. Latencies to different types of seizures, the duration of postseizure immobility, and the number of focal seizure episodes were scored. In the vehicle-treated group, repeated picrotoxin injections led to an increased susceptibility to myoclonic and focal seizures and to decreased duration of postseizure immobility. Naloxone pretreatment significantly decreased the duration of the postseizure akinetic periods in the 1.0- and 10.0-mg/kg groups across all days, suggesting that endogenous opiates are involved in postseizure immobility and that there are interactions between opiate and picrotoxin mechanisms in some seizure-related behaviors. Naloxone did not alter the development of tolerance or sensitivity, indicating that naloxone-insensitive opiate mechanisms or nonopiate mechanisms may be involved in these processes.

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.

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

2009-09-15

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.

Eocene lizard from Germany reveals amphisbaenian origins.

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

2011-05-19

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.

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

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Marzieh Assareh

2010-06-01

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.

Selective impairment of verb processing associated with pathological changes in Brodmann areas 44 and 45 in the motor neurone disease-dementia-aphasia syndrome.

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Bak, T H; O'Donovan, D G; Xuereb, J H; Boniface, S; Hodges, J R

2001-01-01

We report six patients with clinically diagnosed and electrophysiologically confirmed motor neurone disease (MND), in whom communication problems were an early and dominant feature. All patients developed a progressive non-fluent aphasia culminating in some cases in complete mutism. In five cases, formal testing revealed deficits in syntactic comprehension. Comprehension and production of verbs were consistently more affected those that of nouns and this effect remained stable upon subsequent testing, despite overall deterioration. The classical signs of MND, including wasting, fasciculations and severe bulbar symptoms, occurred over the following 6-12 months. The behavioural symptoms ranged from mild anosognosia to personality change implicating frontal-lobe dementia. In three cases, post-mortem examination has confirmed the clinical diagnosis of MND-dementia. In addition to the typical involvement of motor and premotor cortex, particularly pronounced pathological changes were observed in the Brodmann areas 44 (Broca's area) and 45. The finding of a selective impairment of verb/action processing in association with the dementia/aphasia syndrome of MND suggests that the neural substrate underlying verb representation is strongly connected to anterior cortical motor systems.

The role of the cerebellum in the regulation of language functions.

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Starowicz-Filip, Anna; Chrobak, Adrian Andrzej; Moskała, Marek; Krzyżewski, Roger M; Kwinta, Borys; Kwiatkowski, Stanisław; Milczarek, Olga; Rajtar-Zembaty, Anna; Przewoźnik, Dorota

2017-08-29

The present paper is a review of studies on the role of the cerebellum in the regulation of language functions. This brain structure until recently associated chiefly with motor skills, visual-motor coordination and balance, proves to be significant also for cognitive functioning. With regard to language functions, studies show that the cerebellum determines verbal fluency (both semantic and formal) expressive and receptive grammar processing, the ability to identify and correct language mistakes, and writing skills. Cerebellar damage is a possible cause of aphasia or the cerebellar mutism syndrome (CMS). Decreased cerebellocortical connectivity as well as anomalies in the structure of the cerebellum are emphasized in numerous developmental dyslexia theories. The cerebellum is characterized by linguistic lateralization. From the neuroanatomical perspective, its right hemisphere and dentate nucleus, having multiple cerebellocortical connections with the cerebral cortical language areas, are particularly important for language functions. Usually, language deficits developed as a result of a cerebellar damage have subclinical intensity and require applying sensitive neuropsychological diagnostic tools designed to assess higher verbal functions.

[Anxiety disorders in DSM-5: an overview on changes in structure and content].

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Wittchen, H-U; Heinig, I; Beesdo-Baum, K

2014-05-01

The Diagnostic and Statistical Manual of Mental Disorders 5 (DSM-5) "anxiety, obsessive-compulsive spectrum, posttraumatic, and dissociative disorders" work group addressed reconceptualization issues regarding all anxiety-related disorders. Based on systematic literature reviews, reanalyses of available data and evaluation of results following the DSM-5 principles it was decided to rearrange the disorder spectrum into separate groupings for the classical anxiety disorders, trauma- and stressor-related disorders, obsessive-compulsive and related disorders, and dissociative disorders. Among the classical anxiety disorders DSM-5 now also includes selective mutism and separation anxiety disorder. A major change from DSM-IV is a drastically simplified classification of panic disorder and agoraphobia. Both conditions can be separately coded in DSM-5 and the overlap is disclosed by a comorbid double diagnosis. The anxiety disorder criteria have been generally harmonized regarding content and order. It was assured that criteria are applicable to all age, gender and cultural groups. Furthermore, diagnosis-specific and cross-cutting dimensional anxiety scales have been developed to supplement categorical diagnosis which appears to facilitate assessment of severity and course of treatment.

Japanese encephalitis can trigger anti-N-methyl-D-aspartate receptor encephalitis.

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Ma, Jiannan; Zhang, Ting; Jiang, Li

2017-06-01

Japanese encephalitis (JE) is usually a monophasic disease; however, in rare cases, patients with JE may have an early relapse after a partial recovery, giving rise to a biphasic pattern for the disease. In this study, we report three pediatric cases in which post-JE relapse was characterized by movement disorder and/or behavioral problems, and was related to anti-N-methyl-D-aspartate receptor (NMDAR) immunoglobulin G (IgG). Serum and cerebrospinal fluid were examined for anti-NMDAR IgG in three patients who had confirmed JE and then developed relapsing symptoms which were similar to those of anti-NMDAR encephalitis. The main symptoms of the two young children were choreoathetosis, irritability, and sleep disorder; while for the teenager, agitation, mutism, rigidity, and sleep disorder were the main symptoms. Samples of cerebrospinal fluid from all patients were positive for anti-NMDAR IgG, and all patients gradually improved with immunotherapy. Testing for NMDAR antibodies is highly recommend in patients with JE, especially those with a relapsing syndrome involving movement disorder and/or behavioral problems, as these patients may benefit from immunotherapy.

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

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F F Oliveira

2011-01-01

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.

The usefulness of SPECT and MRI in the diagnosis of atypical parkinsonian syndromes

International Nuclear Information System (INIS)

Skogseid, I.M.; Gerdts, R.; Nyberg-Hansen, R.; Rootwelt, K.; Bakke, S.J.

2001-01-01

Clinico-pathological studies have shown that only three out of four patients with parkinsonism have idiopathic Parkinson's disease. In patients with so-called Parkinson plus syndrome, the degeneration in the brain is more widespread and the variety of neurological signs greater than in Parkinson's disease. The differentiation of these syndromes from Parkinson's disease can be difficult. Single photon emission computed tomography (SPECT) and magnetic resonance imaging (MRl) can be of value in the differential diagnosis of parkinsonism. We present three patients with atypical parkinsonism in whom MRI and SPECT with β-CIT and epidepride was performed in addition to the clinical evaluation. The three patients all had a rapidly developing symmetric akinetic-rigid syndrome that responded poorly to levodopa. MRI showed findings regarded as typical for multiple system atrophy in two patients, but only nonspecific findings in the third patient. SPECT with β-CIT showed a pronounced bilateral and relatively symmetric reduction in the striatal dopaminergic activity in all patients. SPECT with epidepride showed a clearly reduced striatal D 2 -receptor binding bilaterally in only one of the patients. In patients with atypical parkinsonism, MRI and SPECT with β-CIT and epidepride can give valuable support to the clinical diagnosis of a Parkinson plus syndrome

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

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GILBERTO LEVY

1999-09-01

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.

Peristalsis gap sign at cine magnetic resonance imaging for diagnosing strangulated small bowel obstruction. Feasibility study

International Nuclear Information System (INIS)

Takahara, Taro; Kwee, T.C.; Haradome, Hiroki

2011-01-01

The aim of this study was to determine the feasibility of cine magnetic resonance imaging (MRI) for diagnosing strangulated small bowel obstruction (SBO). This study included 38 patients with clinically confirmed SBO who had undergone cine MRI. Cine MRI scans were evaluated regarding the presence of the 'peristalsis gap sign' (referring to an akinetic or severely hypokinetic closed loop), indicating strangulation. Computed tomography (CT) was performed in 34 of 38 patients with (n=25) or without (n=9) contrast enhancement. CT images were evaluated using a combination of criteria (presence of hyperattenuation, poor contrast enhancement, mesenteric edema, wall thickening, massive ascites) indicating strangulation. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of cine MRI and CT for the diagnosis of strangulation were calculated and compared using surgical findings and the clinical course as the reference standard. Sensitivity, specificity, PPV, and NPV of cine MRI were 100%, 92.9%, 83.3%, and 100%, respectively; and those of CT (of which 26.5% was performed without contrast enhancement) were 66.7%, 92.0%, 75.0%, and 88.5%, respectively. There was no significant difference in diagnostic accuracy between the two methods (P=0.375). Cine MRI is a feasible and promising technique for diagnosing strangulation. (author)

Brain Perfusion in Corticobasal Syndrome with Progressive Aphasia

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Yoshitake Abe

2016-04-01

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.

Viability of dried filaments, survivability and reproduction under water stress, and survivability following heat and UV exposure in Lyngbya martensiana, Oscillatoria agardhii, Nostoc calcicola, Hormidium fluitans, Spirogyra sp. and Vaucheria geminata

International Nuclear Information System (INIS)

Agrawal, S.C.; Singh, V.

2002-01-01

The aim of our study was to determine how long and to what extent Lyngbya martensiana, Oscillatoria agardhii, Nostoc calcicola, Hormidium fluitans and Vaucheria geminata tolerate dry storage at different temperatures, UV-light radiation and water stress imposed by growing them on media with a high agar content and/or in NaCl-containing liquid media. Dried vegetative filaments of Spirogyra sp., Vaucheria geminata and Nostoc calcicola died within 0,5, 1 and 4 h, respectively; those of Hormidium fluitans, Oscillatoria agardhii and Lyngbya martensiana retained viability for 3, 5 and 10 d, respectively. L. martensiana and O. agardhii tolerated 0.8 mol/L NaCl. The resistance to desiccation in L. martensiana and O. agardhii exhibited similar dependence as that to frost, to heat and UV light. The water stress imposed on growing algae either on high-agar solid media or in NaCl-containing liquid media reduced hormogonium formation in L. martensiana and O. agardhii; hetero-cyst and akinete formation in N. calcicola and fragmentation in H. fluitans. In all studied algae the stress reduced at various levels the survival of vegetative parts. Generally, algal body form and composition rather than habitats seem to decide primarily the level of resistance against various stress conditions

A Nostoc punctiforme sugar transporter necessary to establish a Cyanobacterium-plant symbiosis.

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Ekman, Martin; Picossi, Silvia; Campbell, Elsie L; Meeks, John C; Flores, Enrique

2013-04-01

In cyanobacteria-plant symbioses, the symbiotic nitrogen-fixing cyanobacterium has low photosynthetic activity and is supplemented by sugars provided by the plant partner. Which sugars and cyanobacterial sugar uptake mechanism(s) are involved in the symbiosis, however, is unknown. Mutants of the symbiotically competent, facultatively heterotrophic cyanobacterium Nostoc punctiforme were constructed bearing a neomycin resistance gene cassette replacing genes in a putative sugar transport gene cluster. Results of transport activity assays using (14)C-labeled fructose and glucose and tests of heterotrophic growth with these sugars enabled the identification of an ATP-binding cassette-type transporter for fructose (Frt), a major facilitator permease for glucose (GlcP), and a porin needed for the optimal uptake of both fructose and glucose. Analysis of green fluorescent protein fluorescence in strains of N. punctiforme bearing frt::gfp fusions showed high expression in vegetative cells and akinetes, variable expression in hormogonia, and no expression in heterocysts. The symbiotic efficiency of N. punctiforme sugar transport mutants was investigated by testing their ability to infect a nonvascular plant partner, the hornwort Anthoceros punctatus. Strains that were specifically unable to transport glucose did not infect the plant. These results imply a role for GlcP in establishing symbiosis under the conditions used in this work.

A Nostoc punctiforme Sugar Transporter Necessary to Establish a Cyanobacterium-Plant Symbiosis1[C][W

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Ekman, Martin; Picossi, Silvia; Campbell, Elsie L.; Meeks, John C.; Flores, Enrique

2013-01-01

In cyanobacteria-plant symbioses, the symbiotic nitrogen-fixing cyanobacterium has low photosynthetic activity and is supplemented by sugars provided by the plant partner. Which sugars and cyanobacterial sugar uptake mechanism(s) are involved in the symbiosis, however, is unknown. Mutants of the symbiotically competent, facultatively heterotrophic cyanobacterium Nostoc punctiforme were constructed bearing a neomycin resistance gene cassette replacing genes in a putative sugar transport gene cluster. Results of transport activity assays using 14C-labeled fructose and glucose and tests of heterotrophic growth with these sugars enabled the identification of an ATP-binding cassette-type transporter for fructose (Frt), a major facilitator permease for glucose (GlcP), and a porin needed for the optimal uptake of both fructose and glucose. Analysis of green fluorescent protein fluorescence in strains of N. punctiforme bearing frt::gfp fusions showed high expression in vegetative cells and akinetes, variable expression in hormogonia, and no expression in heterocysts. The symbiotic efficiency of N. punctiforme sugar transport mutants was investigated by testing their ability to infect a nonvascular plant partner, the hornwort Anthoceros punctatus. Strains that were specifically unable to transport glucose did not infect the plant. These results imply a role for GlcP in establishing symbiosis under the conditions used in this work. PMID:23463784

Imaging insights into basal ganglia function, Parkinson's disease, and dystonia.

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Stoessl, A Jon; Lehericy, Stephane; Strafella, Antonio P

2014-08-09

Recent advances in structural and functional imaging have greatly improved our ability to assess normal functions of the basal ganglia, diagnose parkinsonian syndromes, understand the pathophysiology of parkinsonism and other movement disorders, and detect and monitor disease progression. Radionuclide imaging is the best way to detect and monitor dopamine deficiency, and will probably continue to be the best biomarker for assessment of the effects of disease-modifying therapies. However, advances in magnetic resonance enable the separation of patients with Parkinson's disease from healthy controls, and show great promise for differentiation between Parkinson's disease and other akinetic-rigid syndromes. Radionuclide imaging is useful to show the dopaminergic basis for both motor and behavioural complications of Parkinson's disease and its treatment, and alterations in non-dopaminergic systems. Both PET and MRI can be used to study patterns of functional connectivity in the brain, which is disrupted in Parkinson's disease and in association with its complications, and in other basal-ganglia disorders such as dystonia, in which an anatomical substrate is not otherwise apparent. Functional imaging is increasingly used to assess underlying pathological processes such as neuroinflammation and abnormal protein deposition. This imaging is another promising approach to assess the effects of treatments designed to slow disease progression. Copyright © 2014 Elsevier Ltd. All rights reserved.

Imaging insights into basal ganglia function, Parkinson’s disease, and dystonia

Science.gov (United States)

Stoessl, A. Jon; Lehericy, Stephane; Strafella, Antonio P.

2015-01-01

Recent advances in structural and functional imaging have greatly improved our ability to assess normal functions of the basal ganglia, diagnose parkinsonian syndromes, understand the pathophysiology of parkinsonism and other movement disorders, and detect and monitor disease progression. Radionuclide imaging is the best way to detect and monitor dopamine deficiency, and will probably continue to be the best biomarker for assessment of the effects of disease-modifying therapies. However, advances in magnetic resonance enable the separation of patients with Parkinson’s disease from healthy controls, and show great promise for differentiation between Parkinson’s disease and other akinetic-rigid syndromes. Radionuclide imaging is useful to show the dopaminergic basis for both motor and behavioural complications of Parkinson’s disease and its treatment, and alterations in non-dopaminergic systems. Both PET and MRI can be used to study patterns of functional connectivity in the brain, which is disrupted in Parkinson’s disease and in association with its complications, and in other basal-ganglia disorders such as dystonia, in which an anatomical substrate is not otherwise apparent. Functional imaging is increasingly used to assess underlying pathological processes such as neuroinflammation and abnormal protein deposition. This imaging is another promising approach to assess the effects of treatments designed to slow disease progression. PMID:24954673

Changes in the phase and amplitude images in the rehabilitation phase after myocardial infarction

International Nuclear Information System (INIS)

Csernay, L.; Mester, J.; Vidakovich, T.; Rajtar, M.; Pavics, L.; Szasz, K.

1984-01-01

A studing involving patients with completed myocardial infarction, who underwent a 3-week exercise program at a cardiocirculatory rehabilitation center in Southern Hungary, is described. Infarctions were confirmed by the typical clinical and ECG signs and symptoms as well as by 201-T1 imaging at rest. Patients with normal 201-T1 activity distribution were excluded. Three ECG-gated equilibrium radionuclide studies were performed in each case: The first was done on the first day of rehabilitation (at a mean post-infarction interval of 1.5 months); the second study was scheduled 3 weeks after the first on completion of the exercise program and the last 9 weeks after the first (on an outpatient basis). From April 25, 1983 to September 9, 1983 a total of 25 patients were investigated. Of these, 9 had normal 201-T1 images. Of the remaining 16, 9 showed no significant changes of the phase and amplitude images. In 2 cases dyskinesia was found to have been replaced by akinesia, and in another 3 akinesia was replaced by hypokinesia. By contrast, 2 previously akinetic patients became dyskinetic. We expect to increase our patient material to at least 50 cases by the end of 1983 and would like to present our results, illustrating them by some typical examples. (Author)

Non-hyperammonaemic valproate encephalopathy after 20 years of treatment

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Elizabeth Caruana Galizia

2017-01-01

Full Text Available Sodium valproate is a commonly used antiseizure drug with broad indications for different seizuretypes and epilepsy syndromes. Well-recognised side effects include weight gain, tremor, dizziness, and unsteadiness. Non-hyperammonaemic parkinsonism, with or without cognitive impairment, is a rare adverse effect of sodium valproate. We present the case of a sixty year-old lady with a generalized seizure disorder, treated with phenytoin, valproate, lamotrigine and clonazepam. Following withdrawal of phenytoin she developed an akinetic-rigid syndrome, with ataxia and marked cognitive impairment. Extensive investigation failed to identify a cause. Serum ammonia and valproate levels were normal. Hypothesizing this might be valproate encephalopathy, valproate was rapidly substituted with levetiracetam. Her severe motor symptoms resolved within two weeks and cognitive impairment markedly improved. Valproate-induced encephalopathy, with or without hyperammonaemia and liver toxicity are typically recognizable for their temporal relation between the start of therapy with valproate and emergence of the clinical syndrome. Reversible disorders of motor function and cognition attributable to valproate are well described, but few cases have been reported presenting years after starting treatment. Given the insidious progression, delayed onset, lack of association with drug levels or presence of hyperammonaemia, a high index of suspicion is needed to make the diagnosis.

High-dose calcium stimulation test in a case of insulinoma masquerading as hysteria.

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Nakamura, Yoshio; Doi, Ryuichiro; Kohno, Yasuhiro; Shimono, Dai; Kuwamura, Naomitsu; Inoue, Koichi; Koshiyama, Hiroyuki; Imamura, Masayuki

2002-11-01

It is reported that some cases with insulinoma present with neuropsychiatric symptoms and are often misdiagnosed as psychosis. Here we report a case of insulinoma masquerading as hysteria, whose final diagnosis could be made using high-dose calcium stimulation test. A 28-yr-old woman was referred presenting with substupor, mutism, mannerism, restlessness, and incoherence. Laboratory examinations revealed hypoglycemia (33 mg/dL) and detectable insulin levels (9.7 microU/mL), suggesting the diagnosis of insulinoma. However, neither imaging studies nor selective arterial calcium injection (SACI) test with a conventional dose of calcium (0.025 mEq/kg) indicated the tumor. High-dose calcium injection (0.05 mEq/kg) evoked insulin secretion when injected into superior mesenteric artery. A solitary tumor in the head of the pancreas was resected, and her plasma glucose returned to normal. Postoperatively, iv injection of secretin resulted in a normal response of insulin, which was not found preoperatively. This case suggests the usefulness of the SACI test with high-dose of calcium in the case of insulinoma when the standard dose fails to detect such a tumor.

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

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Yiming Yang

2016-12-01

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.

Evaluation of myocardial viability with 99Tcm-tetrofosmin after nitrate administration: comparison with FDG PET imaging

International Nuclear Information System (INIS)

He Wei; Cuocolo, A.

2007-01-01

Objective: The purpose of this study was to assess the relationship between tetrofosmin uptake after nitrate administration and the metabolic activity assessed by 18 F-FDG PET in patients with ischemic left ventricular (LV) dysfunction. Methods: A baseline 99 Tc m -tetrofosmin SPECT at rest and a repeated study after sublingual administration of 10 mg isosorbide dinitrate within two days were undertaken in 36 patients with chronic myocardial infarction and LV dysfunction. All patients underwent metabolic PET imaging with 18 F-FDG in the following week. 99 Tc m -tetrofosmin uptake and metabolic activity in 13 segments of myocardium were measured in every patient. A 55% peak activity on tetrofosmin and 50% peak activity on FDG were used as the differential threshold in evaluating myocardial viability, and the uptakes compared with regional LV function assessed by echocardiography. Results: Fifty-three (40%) of the 131 akinetic or dyskinetic segments had reduced tracer uptake. Of those segments, 14 (26%) segments showed increased tetrofosmin uptake after nitrate intervention (>10% vs baseline), 39 (74%) segments remained no change. The sensitivity and specificity of baseline tetrofosmin SPECT for detecting preserved metabolic activity were 69% and 86%, respectively. After nitrate administration, the sensitivity increased to 81% (P 99 Tc m -tetrofosmin SPECT after nitrate administration may improve the identification of ischemic but still viable myocardium in patients with chronic ischemic LV dysfunction. (authors)

Movement disorders in hereditary ataxias.

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Garcia Ruiz, Pedro J; Mayo, David; Hernandez, Jaime; Cantarero, Susana; Ayuso, Carmen

2002-10-15

Movement disorders are well known features of some dominant hereditary ataxias (HA), specially SCA3/Machado-Joseph disease and dentatorubropallidolusyan atrophy. However, little is known about the existence and classification of movement disorders in other dominant and recessive ataxias. We prospectively studied the presence of movement disorders in patients referred for HA over the last 3 years. Only those patients with a confirmed family history of ataxia were included. We studied 84 cases of HA, including 46 cases of recessive and 38 cases of dominant HA. Thirty out of 46 cases of recessive HA could be classified as: Friedreich ataxia (FA), 29 cases; vitamin E deficiency, 1 case. Twenty-three out of 38 cases of dominant HA could be classified as: SCA 2, 4 cases; SCA 3, 8 cases; SCA 6, 4 cases; SCA 7, 6 cases and SCA 8, 1 case. We observed movement disorders in 20/38 (52%) patients with dominant HA and 25/46 (54%) cases with recessive HA, including 16 patients (16/29) with FA. In general, postural tremor was the most frequent observed movement disorder (27 cases), followed by dystonia (22 cases). Five patients had akinetic rigid syndrome, and in 13 cases, several movement disorders coexisted. Movement disorders are frequent findings in HA, not only in dominant HA but also in recessive HA. Copyright 2002 Elsevier Science B.V.

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

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

2011-10-01

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.

Morphological characterization and molecular fingerprinting of Nostoc strains by multiplex RAPD.

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Hillol, Chakdar; Pabbi, Sunil

2012-01-01

Morphological parameters studied for the twenty selected Nostoc strains were mostly found to be consistent with the earlier reports. But the shape of akinetes observed in this study was a little deviation from the existing descriptions and heterocyst frequency was also found to be different in different strains in spite of growing in the same nitrogen free media. Multiplex RAPD produced reproducible and completely polymorphic amplification profiles for all the strains including some strain specific unique bands which are intended to be useful for identification of those strains. At least one to a maximum of two unique bands was produced by different dual primer combinations. For ten strains out of twenty, strain specific bands were found to be generated. Cluster analysis revealed a vast heterogeneity among these Nostoc strains and no specific clustering based on geographical origin was found except a few strains. It was also observed that morphological data may not necessarily correspond to the genetic data in most of the cases. CCC92 (Nostoc muscorum) and CCC48 (Nostoc punctiforme) showed a high degree of similarity which was well supported by high bootstrap value. The level of similarity of the strains ranged from 0.15 to 0.94. Cluster analysis based on multiplex RAPD showed a good fit revealing the discriminatory power of this technique.

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

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Jane Y Chen

2013-07-01

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.

ST segment elevation after myocardial infarction: Viability or ventricular dysfunction? Comparison with myocardial scintigraphy

International Nuclear Information System (INIS)

Chalela, William Azem; Soares, J. Jr.; Meneghetti, J.C.; Olivera, C.G.; Moffa, P.J.; Falcao, A.M.; Ramires, J.A.F.

2004-01-01

The detection of viable myocardium after myocardial infarction is an important indication for revascularization. We compared exercise-induced ST segment elevation with reversibility at Thallium-201 SPECT scintigraphy and regional wall motion assessment by ventriculography. Thirty two patients with previous myocardial infarction and with left ventricular ejection fraction of < 50% were studied. Patients underwent coronary angiography and Thallium-201 SPECT scintigraphy with re-injection protocol before and after coronary artery bypass graft surgery. Group I comprised 11 patients with ST segment elevation during treadmill stress testing. Group II comprised 21 patients without ST segment elevation. Minimal or moderate hypokinesis was present in 2 patients of Group I and in 4 patients of Group II. Nine patients of Group I and 17 patients of Group II had severe hypokinetic, akinetic or dyskinetic myocardium. Scintigraphy revealed reversibility in the myocardial infarction area in 4 patients from Group I (36.4%) and 11 (52.4%) patients from Group II. Improvement in perfusion after coronary artery bypass grafting was observed in 4 patients from Group I and 8 patients from Group II. Sensitivity, specificity, accuracy, and positive and negative predictive values of ST segment elevation were 33.3, 70.6, 55.2, 44.5 and 60% respectively. It was concluded that exercise-induced ST segment elevation after myocardial infarction is present more frequently in cases of severe regional myocardial dysfunction. (author)

Evaluation of blood signal in cardiac MR imaging using ''black-blood'' technique

International Nuclear Information System (INIS)

Nakanishi, Tadashi; Yamada, Takayuki; Tamura, Akihisa; Miyasaka, Kenji; Kohata, Minako; Ono, Chiaki; Kajima, Toshio; Ito, Katsuhide

1999-01-01

Degradation of image quality encountered in cardiac imaging has been attributed to flowing blood signal in the ventricular cavity. To solve this problem, a sequence in which a pair of selective and non-selective inversion pulse in used for a preparation pulse, has been proposed. However, even with this sequence we frequently observed the signal in the blood pool caused by blood itself rather than blood flow. In this article, we investigated the characteristics of those signals. Five healthy normal volunteers and 13 patients with ischemic heart disease were scanned with a 1.5-tesla MR imager. Breath-hold ECG gated fast spin echo with the pair of inversion pulses was performed to obtain cardiac images with T 2 contrast. Typical blood signal appeared as inhomogeneous high intense band adjacent to inner surface of left ventricular apex. At ventricular base, no such signal was encountered even at akinetic myocardium in patients with old myocardial infarction. This signal was observed in all volunteers and 39% of patients. Decrease of TR resulting from tachycardia tended to reduce the blood signal in the left ventricular cavity. Thicker slice section and selective inversion pulse tended to increase the blood signal. Recognition of the signal is essential to differentiate true myocardial infarcts from blood signal, although bright blood imaging like gradient echo or thinner section can partly be helpful. (author)

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 (Daniel); P.F. Paquier; M.H. Lequin (Maarten); P.G.H. Mulder (Paul)

1999-01-01

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

[A psycholinguistic study in a patient with echolalia].

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Diesfeldt, H F

1986-10-01

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.

Dysgraphia in Patients with Primary Lateral Sclerosis: A Speech-Based Rehearsal Deficit?

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

2008-01-01

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. PMID:19096141

A Peruvian family with a novel PARK2 mutation: Clinical and Pathological Characteristics

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Cornejo-Olivas, Mario; Torres, Luis; Mata, Ignacio F; Mazzetti, Pilar; Rivas, Diana; Cosentino, Carlos; Inca-Martinez, Miguel; Cuba, Juan M; Zabetian, Cyrus P.; Leverenz, James B.

2015-01-01

Background Mutations in PARK2 result in autosomal recessive young onset Parkinson’s disease (YOPD). Although there have been a number of reports on the clinical characteristics of PARK2-related PD, there is limited information available on the associated neuropathologic changes. Design We describe the clinical and pathological characteristics of a Peruvian family with YOPD. The proband and one unaffected sibling were screened for PARK2 dosage and point mutations. One affected sibling had detailed neuropathologic examination. Setting Instituto Nacional de Ciencias Neurologicas (INCN) in Lima, Peru Results The proband and two of her four siblings developed YOPD and both parents were unaffected. The clinical course has been characterized by akinetic-rigid parkinsonism predominantly affecting the lower limbs and dyskinesias. Analysis of PARK2 showed that the proband is compound heterozygous for a novel acceptor splice site mutation in intron 5 (IVS5-1G>A) and an exon 7 deletion. Neuropathologic assessment of an affected sibling revealed severe neuronal loss in the substantia nigra (SN) and loss of tyrosine hydroxylase immunopositive fibers in the striatum. No Lewy body pathology was observed using standard histology or immunohistochemistry for α-synuclein. Conclusions Consistent with most neuropathologic reports of patients with PARK2 mutations, we did not observe Lewy body inclusions, despite marked SN degeneration and severe dopaminergic denervation of the striatum. These data describe a novel splice site mutation and further extend the clinicopathological characterization of PARK2-associated PD. PMID:25817512

Beta oscillations in freely moving Parkinson's subjects are attenuated during deep brain stimulation.

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Quinn, Emma J; Blumenfeld, Zack; Velisar, Anca; Koop, Mandy Miller; Shreve, Lauren A; Trager, Megan H; Hill, Bruce C; Kilbane, Camilla; Henderson, Jaimie M; Brontë-Stewart, Helen

2015-11-01

Investigations into the effect of deep brain stimulation (DBS) on subthalamic (STN) beta (13-30 Hz) oscillations have been performed in the perioperative period with the subject tethered to equipment. Using an embedded sensing neurostimulator, this study investigated whether beta power was similar in different resting postures and during forward walking in freely moving subjects with Parkinson's disease (PD) and whether STN DBS attenuated beta power in a voltage-dependent manner. Subthalamic local field potentials were recorded from the DBS lead, using a sensing neurostimulator (Activa(®) PC+S, Medtronic, Inc., Food and Drug Administration- Investigational Device Exemption (IDE)-, institutional review board-approved) from 15 PD subjects (30 STNs) off medication during lying, sitting, and standing, during forward walking, and during randomized periods of 140 Hz DBS at 0 V, 1 V, and 2.5/3 V. Continuous video, limb angular velocity, and forearm electromyography recordings were synchronized with neural recordings. Data were parsed to avoid any movement or electrical artifact during resting states. Beta power was similar during lying, sitting, and standing (P = 0.077, n = 28) and during forward walking compared with the averaged resting state (P = 0.466, n = 24), although akinetic rigid PD subjects tended to exhibit decreased beta power when walking. Deep brain stimulation at 3 V and at 1 V attenuated beta power compared with 0 V (P closed-loop DBS. © 2015 International Parkinson and Movement Disorder Society.

Comparison of two motor subtype classifications in de novo Parkinson's disease.

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Choi, Seong-Min; Kim, Byeong C; Cho, Bang-Hoon; Kang, Kyung Wook; Choi, Kang-Ho; Kim, Joon-Tae; Lee, Seung-Han; Park, Man-Seok; Kim, Myeong-Kyu; Cho, Ki-Hyun

2018-04-18

Clinical subtypes of Parkinson's disease (PD) have been empirically defined based on the prominent motor symptoms. The aim of this study was to compare the prevalence of non-motor symptoms across PD motor subtypes in patients with PD. A total of 192 patients with de novo PD were included. The patients were classified into the tremor-dominant/mixed/akinetic-rigid (TD/mixed/AR) and tremor-dominant/mixed/postural instability and gait disturbance (TD/mixed/PIGD) subtypes, according to previous reports. In the TD/mixed/AR classification, scores for scales related to motor symptoms and activities of daily living (ADL) were significantly different among the groups, and patients with the AR subtype demonstrated more severe scores than patients with the TD subtype. In the TD/mixed/PIGD classification, age, age at symptom onset, scores on motor-related scales, ADL, and non-motor symptoms were significantly different among the groups. Scores including the modified Hoehn and Yahr stages, the motor and ADL subscores of the Unified Parkinson's Disease Rating Scale, the Beck Depression Inventory, and the Non-Motor Symptom Assessment Scale were significantly different after adjustments for age and age at symptom onset, and patients with the PIGD subtype obtained more severe scores than patients with the TD subtype. The TD/mixed/PIGD classification seems to be more suitable for identifying non-motor abnormalities than the TD/mixed/AR classification. Copyright © 2018 Elsevier Ltd. All rights reserved.

Association between REM sleep behaviour disorder and impulse control disorder in patients with Parkinson's disease.

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Bellosta Diago, E; Lopez Del Val, L J; Santos Lasaosa, S; López Garcia, E; Viloria Alebesque, A

2017-10-01

The relationship between impulse control disorder (ICD) and REM sleep behaviour disorder (RBD) has not yet been clarified, and the literature reports contradictory results. Our purpose is to analyse the association between these 2 disorders and their presence in patients under dopaminergic treatment. A total of 73 patients diagnosed with Parkinson's disease and treated with a single dopamine agonist were included in the study after undergoing clinical assessment and completing the single-question screen for REM sleep behaviour disorder and the short version of the questionnaire for impulsive-compulsive behaviours in Parkinson's disease. Mean age was 68.88 ± 7.758 years. Twenty-six patients (35.6%) were classified as probable-RBD. This group showed a significant association with ICD (P=.001) and had a higher prevalence of non-tremor akinetic rigid syndrome and longer duration of treatment with levodopa and dopamine agonists than the group without probable-RBD. We found a significant correlation between the use of oral dopamine agonists and ICD. Likewise, patients treated with oral dopamine agonists demonstrated a greater tendency toward presenting probable-RBD than patients taking dopamine agonists by other routes; the difference was non-significant. The present study confirms the association between RBD and a higher risk of developing symptoms of ICD in Parkinson's disease. Copyright © 2016 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

Impact of surgery targeting the caudal intralaminar thalamic nuclei on the pathophysiological functioning of basal ganglia in a rat model of Parkinson's disease.

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Kerkerian-Le Goff, Lydia; Bacci, Jean-Jacques; Jouve, Loreline; Melon, Christophe; Salin, Pascal

2009-02-16

There is accumulating evidence that the centre median-parafascicular (CM/Pf) complex of the thalamus is implicated in basal ganglia-related movement disorders and notably in Parkinson's disease. However, the impact of the changes affecting CM/Pf on the pathophysiological functioning of basal ganglia in parkinsonian state remains poorly understood. To address this issue, we have examined the effects of excitotoxic lesion of CM/Pf and of 6-hydroxydopamine-induced lesion of nigral dopamine neurons, separately or in association, on gene expression of markers of neuronal activity in the rat basal ganglia (striatal neuropeptide precursors, GAD67, cytochrome oxidase subunit I) by quantitative in situ hybridization histochemistry. CM/Pf lesion prevented the changes produced by the dopamine denervation in the components of the indirect pathway connecting the striatum to the output structures (striatopallidal neurons, globus pallidus, subthalamic nucleus), and among the output structures, in the entopeduncular nucleus. Preliminary data on the effects of deep brain stimulation of CM/Pf in rats with nigral dopamine lesion show that this surgical approach produces efficient anti-akinetic effect associated with partial reversal of the dopamine lesion-induced increase in striatal preproenkephalin A mRNA levels, a marker of the striatopallidal neurons. These data, which provide substrates for the potential of CM/Pf surgery in the treatment of movement disorders, are discussed in comparison with the effects of lesion or deep brain stimulation of the subthalamic nucleus, the currently preferred target for the surgical treatment of PD.

Neurophysiology and neurochemistry of corticobasal syndrome.

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Murgai, Aditya A; Jog, Mandar S

2018-01-06

Corticobasal syndrome is a rare neurodegenerative disorder, which presents with a progressive, asymmetrical, akinetic rigid syndrome and early cortical signs. However, clinical, pathological, and electrophysiological heterogeneity makes the understanding of this syndrome challenging. Corticobasal syndrome can have various pathological substrates including corticobasal degeneration, Alzheimer's disease, Fronto-temporal degeneration with TDP inclusions, Creutzfeldt-Jakob disease, and progressive supranuclear palsy (PSP). Furthermore, tools such as transcranial magnetic stimulation (TMS) and functional neuroimaging techniques like PET and SPECT have not been adequately used to supplement the clinico-pathological heterogeneity. TMS studies in CBS have revealed changes in cortical excitability and transcortical inhibition. Despite the availability of more than 2 decades, its potential in CBS has not been fully utilized in studying the cortical plasticity and effect of Levodopa on central neurophysiology. PET and SPECT studies in CBS have shown abnormalities in regional glucose metabolism, asymmetrical involvement of presynaptic dopaminergic system, and ascending cholinergic connections to the cortex. While most studies have shown normal D2 receptor-binding activity in striatum of CBS cases, the results have not been unanimous. Functional neuroimaging and TMS studies in CBS have shown the involvement of GABAergic, muscarinic, and dopaminergic systems. In this review, we aim to provide the current state of understanding of central neurophysiology and neurochemistry of CBS using TMS and functional neuroimaging techniques. We also highlight the heterogeneous nature of this disorder and the existing knowledge gaps.

99Tcm-MIBI single photon emission tomography (SPET) for detecting myocardial ischaemia and necrosis in patients with significant coronary artery disease

International Nuclear Information System (INIS)

Sciammarella, M.G.; Fragasso, G.; Gerundini, P.; Maffioli, L.; Cappelletti, A.; Margonato, A.; Savi, A.; Chierchia, S.

1992-01-01

The ability of 99 Tc m -methoxyisobutylisonitrile (MIBI) single photon emission tomography (SPET) to detect myocardial ischaemia and necrosis was assessed in 56 patients with clinically recognised ischaemic heart disease (IHD). All underwent coronary angiography (CA) and left ventriculography (LV). SPET images were obtained at rest and at peak exercise 90 min after injection of 99 Tc m -MIBI. The presence of persistent (P) or reversible (R) perfusion defects (PD) was then correlated to the resting and exercise ECG and to the results of CA and LV. Of the 56 patients, 34 had reversible underperfusion (RPD), 46 persistent underperfusion (PPD) and 31 had both. The occurrence of RPD correlated well with the occurrence of exercise-induced ST segment depression and/or angina (27 patients of 34 patients, 79%) and with the presence of significant coronary artery disease (CAD) (33 of 44, 73%). In 45 of 46 patients (98%) PPD corresponded to akinetic or severely hypokinetic segments (LV) usually explored by ECG leads exhibiting diagnostic Q waves (42 of 46 patients, 91%). The scan was normal both at rest and after stress in four of 11 patients with no CAD, and in two of 45 patients with CAD. Finally, an abnormal resting scan was seen in seven of 11 patients with normal coronary arteries, of whom six had regional wall motion abnormalities. In conclusion, MIBI SPET is a highly reliable technique for assessing the presence and location of myocardial ischaemia and necrosis. (Author)

[sup 99]Tc[sup m]-MIBI single photon emission tomography (SPET) for detecting myocardial ischaemia and necrosis in patients with significant coronary artery disease

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Sciammarella, M.G.; Fragasso, G.; Gerundini, P.; Maffioli, L.; Cappelletti, A.; Margonato, A.; Savi, A.; Chierchia, S. (Istituto Scientifico H San Raffaele, Milan (Italy). Dept. of Nuclear Medicine)

1992-12-01

The ability of [sup 99]Tc[sup m]-methoxyisobutylisonitrile (MIBI) single photon emission tomography (SPET) to detect myocardial ischaemia and necrosis was assessed in 56 patients with clinically recognised ischaemic heart disease (IHD). All underwent coronary angiography (CA) and left ventriculography (LV). SPET images were obtained at rest and at peak exercise 90 min after injection of [sup 99]Tc[sup m]-MIBI. The presence of persistent (P) or reversible (R) perfusion defects (PD) was then correlated to the resting and exercise ECG and to the results of CA and LV. Of the 56 patients, 34 had reversible underperfusion (RPD), 46 persistent underperfusion (PPD) and 31 had both. The occurrence of RPD correlated well with the occurrence of exercise-induced ST segment depression and/or angina (27 patients of 34 patients, 79%) and with the presence of significant coronary artery disease (CAD) (33 of 44, 73%). In 45 of 46 patients (98%) PPD corresponded to akinetic or severely hypokinetic segments (LV) usually explored by ECG leads exhibiting diagnostic Q waves (42 of 46 patients, 91%). The scan was normal both at rest and after stress in four of 11 patients with no CAD, and in two of 45 patients with CAD. Finally, an abnormal resting scan was seen in seven of 11 patients with normal coronary arteries, of whom six had regional wall motion abnormalities. In conclusion, MIBI SPET is a highly reliable technique for assessing the presence and location of myocardial ischaemia and necrosis. (Author).

Augmentative and Alternative Communication (AAC for a patient with a nonfluent/ agrammatic variant of PPA in the mutism stage

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Jolanta Góral-Półrola

2015-12-01

Loss of the ability to communicate with others has a serious impact upon a patient’s quality of life, and often results in withdrawal and an inability to lead an independent life. The introduction of the Augmentative and Alternative Communication (AAC system proves to be a great help, not only for regaining the ability to communicate, but also for the restoration of social bonds. In consequence, the previously mute patient begins to show signs of social cooperation.

Burrowing with a kinetic snout in a snake (Elapidae: Aspidelaps scutatus).

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Deufel, Alexandra

2017-12-01

Of the few elongate, fossorial vertebrates that have been examined for their burrowing mechanics, all were found to use an akinetic, reinforced skull to push into the soil, powered mostly by trunk muscles. Reinforced skulls were considered essential for head-first burrowing. In contrast, I found that the skull of the fossorial shield-nosed cobra (Aspidelaps scutatus) is not reinforced and retains the kinetic potential typical of many non-fossorial snakes. Aspidelaps scutatus burrows using a greatly enlarged rostral scale that is attached to a kinetic snout that is independently mobile with respect to the rest of the skull. Two mechanisms of burrowing are used: (1) anteriorly directed head thrusts from a loosely bent body that is anchored against the walls of the tunnel by friction, and (2) side-to-side shovelling using the head and rostral scale. The premaxilla, to which the rostral scale is attached, lacks any direct muscle attachments. Rostral scale movements are powered by, first, retractions of the palato-pterygoid bar, mediated by a ligament that connects the anterior end of the palatine to the transverse process of the premaxilla and, second, by contraction of a previously undescribed muscle slip of the m. retractor pterygoidei that inserts on the skin at the edge of the rostral scale. In derived snakes, palatomaxillary movements are highly conserved and power prey capture and transport behaviors. Aspidelaps scutatus has co-opted those mechanisms for the unrelated function of burrowing without compromising the original feeding functions, showing the potential for evolution of functional innovations in highly conserved systems. © 2017 Wiley Periodicals, Inc.

Increased pulsatility index supports diagnosis of vascular parkinsonism versus idiopathic Parkinson's disease.

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Caba, L M; Ferrairó, J I T; Torres, I M; Costa, J F V; Muñoz, R B; Martin, A L

2017-12-29

The diagnosis of vascular parkinsonism (VP) is based on a series of clinical criteria and neuroimaging findings. An increase in transcranial Doppler ultrasonography pulsatility index (PI) has been described as a frequent finding in patients with VP. We aimed to confirm this association and to determine the PI value with the highest sensitivity and specificity for diagnosis of VP. PI was determined in all patients admitted to Hospital Universitari i Politècnic La Fe due to parkinsonism between January 2012 and June 2016. We assessed the probability of having VP based on PI values in patients with a definite diagnosis of either VP or idiopathic Parkinson's disease (IPD). A ROC curve was created to determine the PI value with the highest sensitivity and specificity. We assessed a total of 146 patients with suspected parkinsonism; 54 (37%) were diagnosed with IPD and 15 (10%) with VP. Patients with VP were significantly older than those with IPD (mean age of 79 vs 68.5, P=.00144) and had a higher PI (median of 1.29 [IQR: 1.09-1.38] vs 0.96 [IQR: 0.89-1.16], P=.01328). In our sample, a PI of 1.09 conferred 84% sensitivity and 70% specificity. In our series, the PI was significantly higher in patients with VP than in those with IPD. We therefore support the use of transcranial Doppler ultrasonography for the initial assessment of elderly patients with akinetic-rigid syndrome. Copyright © 2017 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

Morphological Diversity and Evolution of the Jugal in Dinosaurs.

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Sullivan, Corwin; Xu, Xing

2017-01-01

In dinosaurs, as in other reptiles, the homologue of the mammalian zygomatic bone is the jugal. The dinosaurian jugal was primitively triradiate, with posterior, dorsal and anterior processes that respectively contacted the quadratojugal, the postorbital, and the maxilla and lacrimal. However, the jugal evolved along different lines in the three major dinosaurian clades. In theropods this cranial element remained relatively conservative in morphology, apart from being reduced to a rod-like structure in most birds and a few non-avians. In sauropodomorphs the jugal eventually became small, plate-like and nearly restricted to the area below the orbit, even being excluded from the ventral margin of the skull in many derived taxa. Among ornithischians the jugal was highly variable, but in many cases became large and/or adorned with ornamental features such as horns, flanges, and rugosities. The jugal does not appear to have been a site of muscle attachment in most non-avian dinosaurs, but represented an important structural element in the akinetic dinosaurian skull. The conspicuous jugal ornaments seen in many ornithischian dinosaurs, like the less striking ones documented in some saurischians, may have played an important role in the social behavior of the species that possessed them. In many cases they have a weapon-like aspect suggesting use in aggressive displays, if not actual combat, adding to the evidence that agonistic behavior was likely widespread among ornithischians in particular. Anat Rec, 300:30-48, 2017. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

The Nostoc punctiforme Genome

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John C. Meeks

2001-12-31

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.

Progressive Stroke-Like Symptoms in a Patient with Sporadic Creutzfeldt-Jakob Disease

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Jukka Lyytinen

2010-03-01

Full Text Available Sporadic Creutzfeldt-Jakob disease (sCJD is a rare neurodegenerative disorder in which accumulation of a pathogenic isoform of prion protein (PrPSc induces neuronal damage with distinct pathologic features. The prognosis of sCJD is devastating: rapid clinical decline is followed by death generally within months after onset of symptoms. The classic clinical manifestations of sCJD are rapidly progressing dementia, myoclonus, and ataxia. However, the spectrum of clinical features can vary considerably. We describe a definite, neuropathologically verified sCJD in a 67-year-old woman who initially presented with progressive stroke-like symptoms: left-sided hemiparesis and ataxia within a few days. The initial brain magnetic resonance imaging (MRI showed bilateral cortical hyperintensity on diffusion-weighted sequences (DWI resembling multiple ischemic lesions. Despite anticoagulation with low-molecular-weight heparin, the patient deteriorated rapidly, became dysphagic and bedridden with myoclonic jerks on her left side extremities correlating with intermittent high-amplitude epileptiform discharges on electroencephalography (EEG. Basal ganglia hyperintense signal changes in addition to cortical ribboning were seen in DWI images of a follow-up MRI. Repeated EEG recordings showed an evolution to periodic sharp wave complexes. Protein 14-3-3 was positive in her cerebrospinal fluid specimen, in addition to an abnormally high total tau level. In the terminal stage the patient was in an akinetic, mutistic state with deteriorating consciousness. She died 19 days after admission to the hospital. Neuropathologic investigation corroborated the clinical diagnosis of sCJD with spongiform degeneration and immunohistochemical demonstration of the deposition of pathologic PrPSc.

Semiology of psychogenic nonepileptic seizures: An international cross-cultural study.

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Asadi-Pooya, Ali A; Valente, Kette; Alessi, Ruda; Tinker, Jennifer

2017-10-01

We compared the semiology of psychogenic nonepileptic seizures (PNES) between patients from the USA and Brazil. This international cross-cultural comparative study may expand understanding of PNES across the borders. We retrospectively investigated all patients with PNES admitted to one epilepsy center in the USA and one in Brazil. We classified their seizures into four classes: generalized motor, akinetic, focal motor, and subjective symptoms. All patients were interviewed by an epileptologist in both countries and were administered psychological assessment measures, including questions about PNES risk factors. For the statistical analyses, we compared patients from the two nations. Eighty-nine patients (49 from the USA and 40 from Brazil) were studied. Patients from the two countries were not significantly different with regard to sex and age, but patients from Brazil had earlier age at onset (26years vs. 34years; P=0.004) and a significantly greater delay in diagnosis (9.9years vs. 5.6years; P=0.001). Some characteristics of PNES were different between the two groups; patients from the USA had generally more seizure types and more often reported subjective seizures (55% in the USA vs. 10% in Brazil; P=0.0001). Clinical and historical characteristics of the patients were not significantly different. Delay in diagnosis of PNES may represent a major factor in resource-limited countries. Large multicenter cross-cultural studies may reveal subtle but significant cross-cultural differences with respect to the semiological, clinical, and historical aspects of PNES; however, patients with PNES share more similarities than differences. Copyright © 2017 Elsevier Inc. All rights reserved.

Studies on 201Th myocardial scintiscanning

International Nuclear Information System (INIS)

Buchner, U.

1979-01-01

The diagnostical evidence of myocardial scintiscanning with thallium-201 was tested on 98 patients with coronary heart disease. 2 mCi thallium-201 were injected into an arm vene and then scintigrams of the heart were registered partly with a scanner, partly with a gamma camera in several views. The healthy myocardium was found in the thallium-201-scintigram to be a rather homogeneous, horeshoe-shaped activity pattern with intramyocardial activity differences of up to 20% of the maximal thallium-201-activity above the myocard which can be declared to be physiological. In dependency on the local blood flow conditions, thallium-201 is stored only in the healthy, but not in the ischaemic or infarcted myocardium. In the scintigram, these regions are seen as regions with reduced radioactivity. A comparison of the localisation of the infarction in the scintigram with those in the electrocardiagram and coronary angiogram showed a good congrucucy. Scintigrams taken at different times after the infarction brought a decrease in the number of diagnosed storage failures, from 90% to 68% in infarctions older than 6 weeks. A scintigraphical differentiation between fresh and old infarctions was not possible. In cases of angiographically established coronary heart disease without infarction, pathological storage reductions were observed. By comparing the findings obtained by scintiscanning with the results of laevocardiography it was seen that hypokinetic regions in the thallium-201-myocardial scintigram showed in only 6% of the cases a pathological storage defect; akinetic, dyskinetic, and aneurysmatic regions, however, were seen in 65% of the cases as clear activity reductions or failures. (orig./MG) [de

Molecular phylogenetics of emydine turtles: taxonomic revision and the evolution of shell kinesis.

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Feldman, Chris R; Parham, James Ford

2002-03-01

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.

Neurodevelopmental disorders: cluster 2 of the proposed meta-structure for DSM-V and ICD-11.

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Andrews, G; Pine, D S; Hobbs, M J; Anderson, T M; Sunderland, M

2009-12-01

DSM-IV and ICD-10 are atheoretical and largely descriptive. Although this achieves good reliability, the validity of diagnoses can be increased by an understanding of risk factors and other clinical features. In an effort to group mental disorders on this basis, five clusters have been proposed. We now consider the second cluster, namely neurodevelopmental disorders. We reviewed the literature in relation to 11 validating criteria proposed by a DSM-V Task Force Study Group. This cluster reflects disorders of neurodevelopment rather than a 'childhood' disorders cluster. It comprises disorders subcategorized in DSM-IV and ICD-10 as Mental Retardation; Learning, Motor, and Communication Disorders; and Pervasive Developmental Disorders. Although these disorders seem to be heterogeneous, they share similarities on some risk and clinical factors. There is evidence of a neurodevelopmental genetic phenotype, the disorders have an early emerging and continuing course, and all have salient cognitive symptoms. Within-cluster co-morbidity also supports grouping these disorders together. Other childhood disorders currently listed in DSM-IV share similarities with the Externalizing and Emotional clusters. These include Conduct Disorder, Attention Deficit Hyperactivity Disorder and Separation Anxiety Disorder. The Tic, Eating/Feeding and Elimination disorders, and Selective Mutisms were allocated to the 'Not Yet Assigned' group. Neurodevelopmental disorders meet some of the salient criteria proposed by the American Psychiatric Association (APA) to suggest a classification cluster.

Clinico-MRI study of hemispheric disorder in long-term follow-up cases of multiple system atrophy

International Nuclear Information System (INIS)

Konagaya, Masaaki; Miwa, Shigeru; Matsuoka, Yukihiko; Konagaya, Yoko

1998-01-01

Twelve cases of multiple system atrophy (MSA) were studied for clinical and MRI findings of the cerebral hemispheric involvement. The subjects consisted of five olivopontocerebellar atrophy (OPCA) type and seven striatonigral degeneration (SND) type. The age at onset was 56.7±8.0 (M±SD) years, duration of illness at the first MRI study 3.2±1.1 years, duration of illness at the last study 8.1±2.2 years, and the following up duration 4.9±2.0 years. The grasping phenomenon was observed in 70% of the cases examined, snout reflex in 80%, slowness of verbal response in 88%, and decrease of spontaneous speech in 100%. Three cases finally fell into the state of mutism. Three out of ten cases were categorized as dementia by HDS-R (Hasegawa Dementia Scale-Revised) test. Besides the progression of the pontocerebellar atrophy and putaminal changes, MRI study revealed progressive frontal lobe atrophy in most cases. At six years after the onset, SND type showed significantly higher incidence of conspicuous frontal lobe atrophy and dilatation of the Sylvian fissure than OPCA type. Cerebral ventricular dilatation was common feature, and atrophy of the temporal and occipital lobes were observed in several cases. We indicated the possible involvement of the cerebral hemisphere, especially the frontal lobe, and higher nervous function in MSA. (author)

Clinico-MRI study of hemispheric disorder in long-term follow-up cases of multiple system atrophy

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Konagaya, Masaaki; Miwa, Shigeru; Matsuoka, Yukihiko [Suzuka National Hospital, Mie (Japan); Konagaya, Yoko

1998-12-01

Twelve cases of multiple system atrophy (MSA) were studied for clinical and MRI findings of the cerebral hemispheric involvement. The subjects consisted of five olivopontocerebellar atrophy (OPCA) type and seven striatonigral degeneration (SND) type. The age at onset was 56.7{+-}8.0 (M{+-}SD) years, duration of illness at the first MRI study 3.2{+-}1.1 years, duration of illness at the last study 8.1{+-}2.2 years, and the following up duration 4.9{+-}2.0 years. The grasping phenomenon was observed in 70% of the cases examined, snout reflex in 80%, slowness of verbal response in 88%, and decrease of spontaneous speech in 100%. Three cases finally fell into the state of mutism. Three out of ten cases were categorized as dementia by HDS-R (Hasegawa Dementia Scale-Revised) test. Besides the progression of the pontocerebellar atrophy and putaminal changes, MRI study revealed progressive frontal lobe atrophy in most cases. At six years after the onset, SND type showed significantly higher incidence of conspicuous frontal lobe atrophy and dilatation of the Sylvian fissure than OPCA type. Cerebral ventricular dilatation was common feature, and atrophy of the temporal and occipital lobes were observed in several cases. We indicated the possible involvement of the cerebral hemisphere, especially the frontal lobe, and higher nervous function in MSA. (author)

[Manneristic catatonia. A psychotropic drug refractory chronic progressive course].

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Stöber, G; Jungkunz, G; Franzek, E; Beckmann, H

1996-07-01

Manneristic catatonia, one form of Leonhard's systematic schizophrenias, is illustrated in nine case notes. The essential syndrome of this rare disorder (described by Leonhard in the preneuroleptic era) consisted in mannerisms and progressive stiffness of psychomotor activity. Mannerisms often developed from obsessive and compulsive ideas; whereas distress disappeared, repetitive behavior developed into a stereotype. Complex movements (e.g. not to shake hands; mutism) became mannerisms. With disease progression stiffness of facial expression and gestures and an impairment of voluntary motor activity became increasingly prominent. There were no signs of (neuroleptic-induced) parkinsonism. Manneristic catatonia affects preponderantly men and exhibits an early age of onset (median: 23 years). In none of the cases a family history of psychiatric illness was noted. Severe obstetric and birth complications as well as the high prevalence of supratentorial and cerebellar CT/MR abnormalities in this patient group point to deviations of prenatal brain maturation. The median yearly dose of neuroleptics was 83.1 g chlorpromazin equivalents. The characteristic psychopathology was not essentially influenced by modern psychopharmacological treatment neither in the beginning nor in the long run irrespective of the time of onset of the disease. Continuous high-dose neuroleptic treatment is not efficacious in this distinct group of systematic schizophrenias. Behavioural training in a rehabilitation unit is the treatment of choice from the early beginning.

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

2015-07-01

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

A transitional snake from the Late Cretaceous period of North America.

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

2012-08-09

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.

The comparative analysis of antiparkinsonian activity of glycine combined with amantadine in conditions of changing neurosynaptic transmission

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Mamchur V.I.

2017-10-01

Full Text Available Parkinson's disease is traditionally viewed as a disease which affects the human motor sphere. Besides motor manifestations in the clinical picture of the disease, non-motor manifestations with dementia as the most common are present. The purpose of the work – experimental evaluation of the possible antiparkinsonian action of glycine in terms of experimental models of Parkinson's disease equivalents (akinetic-rigid and tremor forms on the background of antiparkinsonian correction by amantadine. Methods: catalepsy model (inhibition of dopaminergic transmission, equivalents of hypokinesia and rigidity states and model of arekolyn tremor (activation of cholinergic transmission that corresponds to parkinsonian tremor on the background of amantadine administration (50 mg/kg, glycine (100 mg/kg and 200 mg/kg and their combined introduction. The research results show a positive dynamic in combined using of amantadine with glycine at a dose of 100 mg/kg and 200 mg/kg, which was is determined by the low percentage of animals with symptoms of catalepsy (50-70% with evaluation criteria of 0.5-1.8 points with maximum possible 6 points. Similar results were obtained in terms of activation of the cholinergic system (arekolyn tremor. Glycine at a dose of 100 mg/kg and 200 mg/kg facilitated to optimization of antitremor action of amantadine, that is registered in increased latent period of tremor, reduction of its duration and intensity attenuation almost by 2,1 times in comparison with indicators of the control group. Thus, studied combinations of amantadine with glycine at a dose of 100 mg/kg and 200 mg/kg are promising in studying of their influence on dementia in Parkinson's syndrome, and this study will be continued.

New Perspective on Parkinsonism in Frontotemporal Lobar Degeneration

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Hee Kyung Park

2013-04-01

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.

Morphology and elemental composition of recent and fossil cyanobacteria

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

2005-09-01

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

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; Seppaenen, Marko; Noponen, Tommi

2014-01-01

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. [ 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.)

Bee Venom Alleviates Motor Deficits and Modulates the Transfer of Cortical Information through the Basal Ganglia in Rat Models of Parkinson's Disease.

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Maurice, Nicolas; Deltheil, Thierry; Melon, Christophe; Degos, Bertrand; Mourre, Christiane; Amalric, Marianne; Kerkerian-Le Goff, Lydia

2015-01-01

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.

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

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Wen-yan eKang

2014-06-01

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.

99Tcm-MIBI single photon emission tomography (SPET) for detecting myocardial ischaemia and necrosis in patients with significant coronary artery disease.

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Sciammarella, M G; Fragasso, G; Gerundini, P; Maffioli, L; Cappelletti, A; Margonato, A; Savi, A; Chierchia, S

1992-12-01

The ability of 99Tcm-methoxyisobutylisonitrile (MIBI) single photon emission tomography (SPET) to detect myocardial ischaemia and necrosis was assessed in 56 patients (45 male, 11 female, aged 55 +/- 5 years), with clinically recognized ischaemic heart disease (IHD). All underwent coronary angiography (CA) and left ventriculography (LV). SPET images were obtained at rest and at peak exercise (Modified Bruce) 90 min after injection of 99Tcm-MIBI (650-850 MBq). Data were acquired in 30 min over 180 degrees (from 45 degrees RAO to 45 degrees LPO) with no correction for attenuation, using a 64 x 64 matrix. The presence of persistent (P) or reversible (R) perfusion defects (PD) was then correlated to the resting and exercise ECG and to the results of CA and LV. Of the 56 patients, 34 had reversible underperfusion (RPD), 46 persistent underperfusion (PPD) and 31 had both. The occurrence of RPD correlated well with the occurrence of exercise-induced ST segment depression and/or angina (27 patients of 34 patients, 79%) and with the presence of significant coronary artery disease (CAD) (33 of 44, 73%). In 45 of 46 patients (98%) PPD corresponded to akinetic or severely hypokinetic segments (LV) usually explored by ECG leads exhibiting diagnostic Q waves (42 of 46 patients, 91%). The scan was normal both at rest and after stress in four of 11 patients with no CAD, and in two of 45 patients with CAD. Finally, an abnormal resting scan was seen in seven of 11 patients with normal coronary arteries, of whom six had regional wall motion abnormalities.(ABSTRACT TRUNCATED AT 250 WORDS)

Contribution of contractile state of the non-infarcted area to global ventricular performance after acute myocardial infarction

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Nishimura, Tsunehiko; Yasuda, Tsunehiro; Gold, H.K.; Leinbach, R.C.; Boucher, C.A.; McKusick, K.A.; Strauss, H.W.

1986-01-01

To evaluate the regional contractile state of the non-infarcted zone and to determine the contribution of this area to left ventricular (LV) performance, 112 patients (42 anterior and 70 inferior infarction) with their first acute myocardial infarction were investigated by radionuclide ventriculography at admission and 10 days after admission. Wall motion at the non-infarcted area was defined as hyperkinetic, normal, or hypokinetic, if radial chord shortening had above normal, normal, or below normal values, respectively, by quantitative wall motion analysis. Hyperkinetic, normal, and hypokinetic wall motion of the non-infarcted area were observed in three (7 %), 12 (29 %), and 27 (64 %) patients in anterior infarction and 14 (20 %), 28 (40 %), and 28 (40 %) in inferior infarction, respectively. In the patients with hypokinetic wall motion at the non-infarcted area, the infarct involved more than 30 % of the left ventricle manifesting akinetic contractile segment (ACS), radial chord shortening in the infarcted area was severely depressed, and the incidence of multi-vessel involvement was higher compared with those with hyperkinetic or normal wall motion. In serial measurements, radial chord shortening in the infarcted and non-infarcted area, percent ACS, left ventricular ejection fraction, and left ventricular end-diastolic volume index did not change significantly from acute to follow-up study in any group. In conclusion, our data indicated that the non-infarcted area following acute infarction had various contractile states and these conditions were determined primarily by the severity and extent of infarct and underlying coronary artery disease. Furthermore, the contractile state of the non-infarcted area has a supplemental role in determination of LV function following acute infarction. (author)

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

2002-03-01

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.

Prevalence and clinical significance of residual myocardial ischemia 2 weeks after uncomplicated non-Q wave infarction: a prospective natural history study

International Nuclear Information System (INIS)

Gibson, R.S.; Beller, G.A.; Gheorghiade, M.; Nygaard, T.W.; Watson, D.D.; Huey, B.L.; Sayre, S.L.; Kaiser, D.L.

1986-01-01

Despite having smaller infarct size and better left ventricular function, patients with non-Q wave myocardial infarction (NQMI) appear to have an unexpectedly high long-term mortality that is ultimately comparable to that of patients with Q-wave myocardial infarction (QMI). Patients with NQMI may lose their initial prognostic advantage because there is more viable tissue in the perfusion zone of the infarct-related vessel, rendering myocardium more prone to reinfarction. We tested this hypothesis in a prospective study of 241 consecutive patients 65 years of age or younger with acute uncomplicated myocardial infarction confirmed by creatine kinase levels (MB fraction). All patients received customary care and none underwent thrombolytic therapy or emergency angioplasty. Predischarge coronary angiography, radionuclide ventriculography, 24 hr Holter monitoring, and quantitative thallium-201 scintigraphy during treadmill exercise were performed 10 +/- 3 days after infarction. Infarcts were designated as QMI (n = 154) or NQMI (n = 87) by accepted criteria applied to serial electrocardiograms obtained on days 1, 2, 3, and 10. The baseline Norris coronary prognostic index, angiographic jeopardy scores, and prevalence of Lown grade ventricular arrhythmias were similar between groups despite evidence for less necrosis with NQMI vs QMI, reflected by lower peak creatine kinase levels (520 vs 1334 IU/liter; p = .0001, 4 hr sampling), higher resting left ventricular ejection fraction (53% vs 46%; p = .0001), fewer akinetic or dyskinetic segments (1.2 vs 2.4; p = .0001), and fewer persistent 201 Tl defects in the infarct zone. Patients with NQMI also had more patent infarct-related vessels and a shorter time from onset of infarction to peak creatine kinase level

The 7q11.23 Microduplication Syndrome: A Clinical Report with Review of Literature

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

2016-01-01

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. PMID:27617154

Clinical manifestations of Waardenburg syndrome in a male adolescent in Mali, West Africa.

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Imperato, Pascal James; Imperato, Gavin H

2015-02-01

Waardenburg syndrome (WS) is a genetic disorder of which there are four distinct types. These four types are differentiated by the physical defects which they produce. Presented here is the case of a 13-year-old boy with WS Type I who was observed and physically assessed in Mali, West Africa in 1969. His physical findings included a bright blue coloring to the irises of the eyes, profound sensorineural deafness, mutism, dystopia canthorum (lateral displacement of the inner canthi of the eyes), broad nasal root, bushy eyebrows, and scaphoid deformities of the supraorbital portions of the frontal bone. Because family members were not available for interviews or physical examinations, it was not possible to determine if this patient was suffering from a congenital form of the disorder or from a spontaneous mutation. Given the patient's then location in a remote rural area of Mali where electricity was absent, it was not possible to perform additional diagnostic tests. The patient described here is the first with WS in Mali, West Africa to have been medically observed and evaluated and later documented in the medical literature. A second case of the syndrome in Mali was described in the medical literature in 2011 in an 18-month-old infant who did not have sensorineural hearing loss, but who did have a bilateral cleft lip. An historical overview of WS is presented along with details concerning the characteristics of the four types of the disorder.

Anti-N-methyl-D-aspartate receptor-mediated encephalitis in infants and toddlers: case report and review of the literature.

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Goldberg, Ethan M; Titulaer, Maarten; de Blank, Peter M; Sievert, Angela; Ryan, Nicole

2014-02-01

Anti-N-methyl-D-aspartate receptor encephalitis is an increasingly well-recognized inflammatory encephalitis in children and adults. We report a previously healthy 21-month-old girl who presented with behavioral change, self-mutilatory behavior, and echolalia. Over the ensuing weeks, symptoms progressed to include unilateral upper extremity dystonia, gait impairment, dysphagia, and mutism. Magnetic resonance imaging of the brain showed a tiny area of signal abnormality in the subcortical white matter, but was otherwise normal. Continuous video electroencephalography showed slowing of the background rhythm, but was without epileptiform discharges. Lumbar puncture showed a mild pleocytosis of mixed cellularity; bacterial culture and testing for various viral encephalitides were negative. Serum and cerebrospinal fluid was positive for autoantibodies directed against the N-methyl-D-aspartate receptor, and she was diagnosed with anti-N-methyl-D-aspartate receptor encephalitis. The patient was successfully treated with a regimen of immunotherapy that included dexamethasone, intravenous immunoglobulin, and rituximab. One year after initial presentation, the patient remained symptom-free. We further review the clinical characteristics, results of diagnostic studies, treatment, and outcome of infants and toddlers diagnosed with anti-N-methyl-D-aspartate receptor encephalitis that have been previously reported in the literature. Anti-N-methyl-D-aspartate receptor encephalitis is relatively common among infants and toddlers and often presents with a pattern of defining characteristics in this age group, particularly the absence of associated tumor. Copyright © 2014 Elsevier Inc. All rights reserved.

Social anxiety disorder: questions and answers for the DSM-V.

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Bögels, Susan M; Alden, Lynn; Beidel, Deborah C; Clark, Lee Anna; Pine, Daniel S; Stein, Murray B; Voncken, Marisol

2010-02-01

This review evaluates the DSM-IV criteria of social anxiety disorder (SAD), with a focus on the generalized specifier and alternative specifiers, the considerable overlap between the DSM-IV diagnostic criteria for SAD and avoidant personality disorder, and developmental issues. A literature review was conducted, using the validators provided by the DSM-V Spectrum Study Group. This review presents a number of options and preliminary recommendations to be considered for DSM-V. Little supporting evidence was found for the current specifier, generalized SAD. Rather, the symptoms of individuals with SAD appear to fall along a continuum of severity based on the number of fears. Available evidence suggested the utility of a specifier indicating a "predominantly performance" variety of SAD. A specifier based on "fear of showing anxiety symptoms" (e.g., blushing) was considered. However, a tendency to show anxiety symptoms is a core fear in SAD, similar to acting or appearing in a certain way. More research is needed before considering subtyping SAD based on core fears. SAD was found to be a valid diagnosis in children and adolescents. Selective mutism could be considered in part as a young child's avoidance response to social fears. Pervasive test anxiety may belong not only to SAD, but also to generalized anxiety disorder. The data are equivocal regarding whether to consider avoidant personality disorder simply a severe form of SAD. Secondary data analyses, field trials, and validity tests are needed to investigate the recommendations and options.

[Recovery of consciousness: process-oriented approach].

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Gusarova, S B

2014-01-01

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.

Differential diagnosis at admission between Takotsubo cardiomyopathy and acute apical-anterior myocardial infarction in postmenopausal women.

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Zorzi, Alessandro; Baritussio, Anna; ElMaghawry, Mohamed; Siciliano, Mariachiara; Migliore, Federico; Perazzolo Marra, Martina; Iliceto, Sabino; Corrado, Domenico

2016-08-01

Takotsubo cardiomyopathy (TTC) typically affects postmenopausal women and clinically presents with chest pain, ST-segment elevation, elevated cardiac enzymes and apical left ventricular (LV) wall motion abnormalities that mimic 'apical-anterior' acute myocardial infarction (AMI). This study assessed whether at-admission clinical evaluation helps in differential diagnosis between the two conditions. The study compared at-admission clinical, electrocardiographic (ECG) and echocardiographic findings of 31 women (median age 67 years, interquartile range (IQR) 62-76) with typical TTC and 30 women (median age 73 years, IQR 61-81) with apical-anterior AMI due to acute occlusion of the mid/distal left anterior descending coronary artery. Women with TTC significantly more often showed PR-segment depression (62% versus 3%, p<0.001), J-waves (26% versus 3%, p=0.03), maximum ST-segment elevation ⩽2 mm (84% versus 37%, p<0.001) and ST-segment elevation in lead II (42% versus 10%, p=0.01) than those with AMI. At multivariate analysis, PR-segment depression (odds ratio (OR)=37.2, 95% confidence interval (CI)=3.4-424, p=0.002) and maximum ST-segment elevation ⩽2 mm (OR=11.1, 95% CI=1.7-99.4, p=0.01) remained the only independent predictors of TTC and the co-existence of both parameters excluded AMI with a 100% specificity. The two groups did not differ with regard to age, first troponin-I value, echocardiographic LV ejection fraction and distribution of hypo/akinetic LV segments. At-admission electrocardiogram (but no clinical, laboratory and echocardiographic features) allows differential diagnosis between TTC and apical-anterior AMI in postmenopausal women. The combination of PR-segment depression and mild (⩽2 mm) ST-segment elevation predicted TTC with greater accuracy than traditional parameters such as localisation of ST-segment elevation and reciprocal ST-segment depression. © The European Society of Cardiology 2015.

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

International Nuclear Information System (INIS)

Jeong, Young Jin; Kang, Do Young; Park, Kyung Won; Kim, Jae Woo

2004-01-01

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

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)

2004-07-01

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.

Dobutamine cine magnetic resonance imaging after myocardial infarction

International Nuclear Information System (INIS)

Giovagnoni, A.; Ligabue, G.; Romagnoli, R.; Reggio Emilia Univ., Reggio Emilia; Rossi, R.; Muia, N.; Modena, M.G.; Reggio Emilia Univ.

1999-01-01

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, 15γ/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 [it

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)

2014-10-15

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.)

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.

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)

1997-06-01

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.)

USE OF THREE-COMPONENT LEVODOPA (LEVODOPA/CARBIDOPA/ENTACAPONE TO CORRECT NOCTURNAL SYMPTOMS OF PARKINSON,S DISEASE

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T. K. Kulua

2015-01-01

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. 

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

2012-10-01

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

Viability after myocardial infarction: can it be assessed within five minutes by low-dose dynamic iodine-123-iodophenylpentadecanoic acid imaging with a multicrystal gamma camera?

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Murray, G L; Schad, N; Bush, A J

1997-04-01

Although positron emission tomography (PET) assesses myocardial viability (V) accurately, a rapid, inexpensive substitute is needed. Therefore, the authors developed a low-dose (1 mCi) Iodine-123-Iodophenylpentadecanoic Acid (IPPA) myocardial viability scan requiring analysis of only the first three minutes of data acquired at rest with a standard multicrystal gamma camera. Twenty-one patients > 2 weeks after myocardial infarction (MI) (24 MIs, 10 anterior, 14 inferoposterior, 21 akinetic or dyskinetic) had cardiac catheterization and resting IPPA imaging. V was determined by either transmural myocardial biopsy during coronary bypass surgery (12 patients, 14 MIs) or reinjection tomographic thallium scan (9 patients, 10 MIs), and 50% of MIs were viable. The IPPA variables analyzed were: time to initial left ventricular (LV) uptake in the region of interest (ROI), the ratio of three-minute uptake in the ROI to three-minute LV uptake, three-minute clearing (counts/pixel) in the ROI (decrease in IPPA after initial uptake), and three-minute accumulation (increase in IPPA after initial uptake) in the ROI. Rules for detecting V were generated and applied to 10 healthy volunteers to determine normalcy. While three-minute uptake in nonviable MIs was only 67% of volunteers (P or = 13.5 counts/pixel in 10/12 (83%) of viable MIs, and IPPA accumulation > or = 6.75 counts/pixel identified one more viable MI, for a sensitivity for V of 11/12 (92%), with a specificity of 11/12 (92%), and a 100% normalcy rate. The authors conclude low-dose IPPA (five-minute acquisition with analysis of the first three minutes of data) has potential for providing rapid, inexpensive V data after MI. Since newer multicrystal cameras are mobile, IPPA scans can be done in emergency rooms or coronary care units generating information that might be useful in decisions regarding thrombolysis, angioplasty, or bypass surgery.

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)

2007-07-15

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.)

The story of iodine deficiency: An international challenge in nutrition

International Nuclear Information System (INIS)

Hetzel, B.S.

1989-01-01

Iodine deficiency is a risk factor for the growth and development of up to 800 million people living in iodine deficient environments throughout the world. The effects on growth and development, called the iodine deficiency disorders (IDD), comprise goiter, stillbirths and miscarriages, neonatal and juvenile thyroid deficiency, dwarfism, mental defects, deaf mutism, and spastic weakness and paralysis, as well as lesser degrees of loss of physical and mental function. All these effects are due to inadequate thyroid hormone production because iodine is an essential constituent of the thyroid hormone. In the West, IDD has been largely eliminated by the addition of iodine to the diet through iodized salt or through changes in food distribution and technology. IDD still persists in certain areas of Europe where these dietary changes have not occurred. In the Third World, IDD is a major problem in many countries with large populations, such as China, India, Indonesia, Nigeria, and Zaire. In these and other Third World countries, IDD is a significant barrier to social and economic progress which can be removed by correction of the deficiency. This book shows that elimination of iodine deficiency is feasible within the next decade, only requiring a modest financial and technical effort from the West. Part 1 reviews IDD in man and animals. Part 2 discusses the control of iodine deficiency disorders through iodine supplementation, and considers action at the national and international level. Part 3 presents a global review of the status of IDD control. There is a brief conclusion on the way forward to successful control programs

Longitudinal MRI assessment: the identification of relevant features in the development of Posterior Fossa Syndrome in children

Science.gov (United States)

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

2015-03-01

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.

The story of iodine deficiency: An international challenge in nutrition

Energy Technology Data Exchange (ETDEWEB)

Hetzel, B.S.

1989-01-01

Iodine deficiency is a risk factor for the growth and development of up to 800 million people living in iodine deficient environments throughout the world. The effects on growth and development, called the iodine deficiency disorders (IDD), comprise goiter, stillbirths and miscarriages, neonatal and juvenile thyroid deficiency, dwarfism, mental defects, deaf mutism, and spastic weakness and paralysis, as well as lesser degrees of loss of physical and mental function. All these effects are due to inadequate thyroid hormone production because iodine is an essential constituent of the thyroid hormone. In the West, IDD has been largely eliminated by the addition of iodine to the diet through iodized salt or through changes in food distribution and technology. IDD still persists in certain areas of Europe where these dietary changes have not occurred. In the Third World, IDD is a major problem in many countries with large populations, such as China, India, Indonesia, Nigeria, and Zaire. In these and other Third World countries, IDD is a significant barrier to social and economic progress which can be removed by correction of the deficiency. This book shows that elimination of iodine deficiency is feasible within the next decade, only requiring a modest financial and technical effort from the West. Part 1 reviews IDD in man and animals. Part 2 discusses the control of iodine deficiency disorders through iodine supplementation, and considers action at the national and international level. Part 3 presents a global review of the status of IDD control. There is a brief conclusion on the way forward to successful control programs.

Frontotemporal dementia with trans-activation response DNA-binding protein 43 presenting with catatonic syndrome.

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Watanabe, Ryohei; Kawakami, Ito; Onaya, Mitsumoto; Higashi, Shinji; Arai, Nobutaka; Akiyama, Haruhiko; Hasegawa, Masato; Arai, Tetsuaki

2017-11-07

Catatonia is a clinical syndrome characterized by symptoms such as immobility, mutism, stupor, stereotypy, echophenomena, catalepsy, automatic obedience, posturing, negativism, gegenhalten and ambitendency. This syndrome occurs mostly in mood disorder and schizophrenic patients, and is related to neuronal dysfunction involving the frontal lobe. Some cases of frontotemporal dementia (FTD) with catatonia have been reported, but these cases were not examined by autopsy. Here, we report on a FTD case which showed catatonia after the first episode of brief psychotic disorder. At the age of 58, the patient had a sudden onset of disorganized behavior and meaningless speech. Psychotropic drugs were effective for catatonic symptoms. However, after remission apathy, hyperorality, socially inappropriate behavior, hoarding, and an instinctive grasp reaction appeared and persisted. Brain MRI showed significant atrophy of the bilateral fronto-temporal lobes. A neuropathological examination revealed extensive trans-activation response DNA-binding protein 43 (TDP-43) positive neurocytoplasmic inclusions and dystrophic neurites in the brain, including the cerebral cortex, basal ganglia, and brainstem. Pathological diagnosis was frontotemporal lobar degeneration (FTLD) with TDP-43 (FTLD-TDP) type C, which was also confirmed by the band pattern of C-terminal fragments of TDP-43 on western blotting of sarkosyl-insoluble fractions extracted from the frozen brain. Dysfunction of the thalamus, globus pallidus, supplementary motor area, amygdala and cingulate cortex have been said to be related to the catatonic syndrome. In this case, these areas were affected, showing abnormal TDP-43-positive structures. Further studies are expected to confirm further clinical - pathological correlations to FTLD. © 2017 Japanese Society of Neuropathology.

Children with 7q11.23 Duplication Syndrome: Psychological Characteristics

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Mervis, Carolyn B.; Klein-Tasman, Bonita P.; Huffman, Myra J.; Velleman, Shelley L.; Pitts, C. Holley; Henderson, Danielle R.; Woodruff-Borden, Janet; Morris, Colleen A.; Osborne, Lucy R.

2015-01-01

To begin to delineate the psychological characteristics associated with classic 7q11.23 duplication syndrome (duplication of the classic Williams syndrome region; hereafter classic Dup7), we tested 63 children with classic Dup7 aged 4–17 years. Sixteen toddlers aged 18–45 months with classic Dup7 and 12 adults identified by cascade testing also were assessed. For the child group, median General Conceptual Ability (similar to IQ) on the Differential Ability Scales-II was 85.0 (low average), with a range from severe disability to high average ability. Median reading and mathematics achievement standard scores were at the low average to average level, with a range from severe impairment to high average or superior ability. Adaptive behavior was considerably more limited; median Scales of Independent Behavior—Revised Broad Independence standard score was 62.0 (mild impairment), with a range from severe adaptive impairment to average adaptive ability. Anxiety disorders were common, with 50.0% of children diagnosed with Social Phobia, 29.0% with Selective Mutism, 12.9% with Separation Anxiety Disorder, and 53.2% with Specific Phobia. In addition, 35.5% were diagnosed with Attention Deficit/Hyperactivity Disorder and 24.2% with Oppositional Defiant Disorder or Disruptive Behavior Disorder-Not Otherwise Specified. 33.3% of the children screened positive for a possible Autism Spectrum Disorder and 82.3% were diagnosed with Speech Sound Disorder. We compare these findings to previously reported results for children with Williams syndrome and argue that genotype/phenotype studies involving the Williams syndrome region offer important opportunities to understand the contribution of genes in this region to common disorders affecting the general population. PMID:25900101

Paraneoplastic neurological disorders in children with benign ovarian tumors.

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Hsu, Mei-Hsin; Huang, Chao-Ching; Hung, Pi-Lien; Huang, Hsiu-Mei; Huang, Li-Tung; Huang, Chao-Cheng; Sheen, Jiunn-Ming; Huang, Song-Chei; Chang, Ying-Chao

2014-03-01

Paraneoplastic neurological diseases (PND) are rare, but potentially treatable disorders. Paraneoplastic encephalitis is rapidly emerging as an important but likely under-recognized condition in children. The aim of this study was to assess the prevalence and spectrum of PND in children with benign ovary tumor and the long-term outcome. We retrospectively reviewed the charts of all female patients below 18years of age diagnosed with a benign ovarian tumor proven by pathology between January 1993 and December 2010. All the clinical symptoms developed within 5years of tumor diagnosis and the related investigations were recorded. There were total 133 children and adolescents with benign ovarian tumors, mostly mature teratoma. Six patients (4.5%) had neuropsychiatric manifestations and all but one were beyond age 10years. The most common neuropsychiatric presentations were depression or low mood (84%), headache (50%), mutism (50%), hypoventilation (50%), seizures (30%), hallucination (30%), vomiting and hypersalivation (30%). Three patients (2.2%) had serious PND including acute disseminated encephalomyelitis in 1 and anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis in 2. Although all of three improved after tumor removal, one without immunotherapy had neurological sequelae and prolonged ICU stay. The prevalence of PND in benign ovary tumor is not so uncommon in children. It is important to survey ovary tumors in female adolescents with subacute presentation of multiple-level involvement of neuraxis where no clear alternate diagnosis is possible. Treatment of serious PND associated with ovary tumors should include immunotherapy in addition to tumor removal. Copyright © 2013 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Symptom profile and short term outcome of catatonia: an exploratory clinical study.

Science.gov (United States)

Worku, Benyam; Fekadu, Abebaw

2015-07-22

Catatonia is a potentially life-threatening but treatable neuropsychiatric condition. Although considered more common in low income countries, data is particularly sparse in these settings. In this study we explore the symptomatology, treatment, and short-term outcome of catatonia in Ethiopia, a low income country. The study was a prospective evaluation of patients admitted with a DSM-IV diagnosis of catatonia. Diagnosis of Catatonia and its severity were further assessed with the Bush-Francis Catatonia Rating Scale (BFCRS). Twenty participants, 5 male and 15 female, were included in the study: 15 patients (75 %) had underlying mood disorders, 4 patients (20 %) had schizophrenia and 1 patient (5 %) had general medical condition. The most common catatonic symptoms, occurring in over two-thirds of participants, were mutism, negativism, staring and immobility (stupor). Eighteen (90 %) of the twenty patients were on multiple medications. Antipsychotics were the most commonly prescribed medications. ECT was required in seven patients (35.0 %). Dehydration, requiring IV rehydration, and infections were the most important complications ascribed to the catatonia. These occurred in seven patients (25 %). Almost all patients (n = 19/20) were discharged with significant improvement. This study supports the growing consensus that catatonia is most often associated with mood disorders. Overall prognosis appears very good although the occurrence of life-threatening complications underlines the serious nature of catatonia. This has implication for "task-shifted" service scale up plans, which aim to improve treatment coverage by training non-specialist health workers to provide mental health care in low income countries. Further larger scale studies are required to clarify the nature and management, as well as, service requirements for catatonia.

Children with 7q11.23 duplication syndrome: psychological characteristics.

Science.gov (United States)

Mervis, Carolyn B; Klein-Tasman, Bonita P; Huffman, Myra J; Velleman, Shelley L; Pitts, C Holley; Henderson, Danielle R; Woodruff-Borden, Janet; Morris, Colleen A; Osborne, Lucy R

2015-07-01

To begin to delineate the psychological characteristics associated with classic 7q11.23 duplication syndrome (duplication of the classic Williams syndrome region; hereafter classic Dup7), we tested 63 children with classic Dup7 aged 4-17 years. Sixteen toddlers aged 18-45 months with classic Dup7 and 12 adults identified by cascade testing also were assessed. For the child group, median General Conceptual Ability (similar to IQ) on the Differential Ability Scales-II was 85.0 (low average), with a range from severe disability to high average ability. Median reading and mathematics achievement standard scores were at the low average to average level, with a range from severe impairment to high average or superior ability. Adaptive behavior was considerably more limited; median Scales of Independent Behavior-Revised Broad Independence standard score was 62.0 (mild impairment), with a range from severe adaptive impairment to average adaptive ability. Anxiety disorders were common, with 50.0% of children diagnosed with Social Phobia, 29.0% with Selective Mutism, 12.9% with Separation Anxiety Disorder, and 53.2% with Specific Phobia. In addition, 35.5% were diagnosed with Attention Deficit/Hyperactivity Disorder and 24.2% with Oppositional Defiant Disorder or Disruptive Behavior Disorder-Not Otherwise Specified. 33.3% of the children screened positive for a possible Autism Spectrum Disorder and 82.3% were diagnosed with Speech Sound Disorder. We compare these findings to previously reported results for children with Williams syndrome and argue that genotype/phenotype studies involving the Williams syndrome region offer important opportunities to understand the contribution of genes in this region to common disorders affecting the general population. © 2015 Wiley Periodicals, Inc.

Case series of eosinophilic meningoencephalitis from South India

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Parameswaran K

2006-01-01

Full Text Available Eosinophilic meningoencephalitis (EM is a rare type of meningoencephalitis. The objective of this report is to describe a series of EM identified in a specific geographic area over a short period of time. Materials and Methods: This series of cases are described from a neurological center in Central Kerala occuring in the period between February 2004 and June 2006. Results: During this period we had identified ten patients (eight males and two females with EM. Their mean age was 37.1 years (range 15-60 years. Main symptomatologies were fever, severe headache, body pain, abdominal pain and arthralgia. One patient was in akinetic rigid state with coma. All patients had peripheral eosinophilia. The cerebrospinal fluid (CSF of all patients showed eosinophilic pleocytosis. The mean CSF white cell count was 588 cells. CSF differential count showed 50-70% eosinophils. CSF glucose levels were normal but proteins were markedly raised (mean CSF protein was 180 mg/dl. MRI brain showed T2 hyperintensities diffusely in periventricular white matter in the comatose patient. Contrast enhanced CT scan of the brain was normal in others. All eight male patients gave history of eating "raw flesh of Monitor Lizard" (Iguana some three to fourteen days prior to the onset of symptoms. There was no such history for the female patients. Considering the history of exposure and eosinophilic meningitis we suspected a meningoencephalitis with Angiostrongylus cantonensis and treated them with albendazole, steroid and other supportive measures. All of them recovered. Conclusion: Eosinophilic meningitis (EM is a rare condition and in this locality, a CNS infection with Agiostrongylus cantonensis is highly likely. AC is a parasite in monitor lizard. Human infection occurs from consumption of uncooked flesh or blood of infected lizards. Physicians need to maintain a high index of suspicion and enquire for any exposure to uncooked meat or blood of monitor lizard when faced with EM

Characterization of nociceptive response to chemical, mechanical, and thermal stimuli in adolescent rats with neonatal dopamine depletion.

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Ogata, M; Noda, K; Akita, H; Ishibashi, H

2015-03-19

Rats with dopamine depletion caused by 6-hydroxydopamine (6-OHDA) treatment during adulthood and the neonatal period exhibit akinetic motor activity and spontaneous motor hyperactivity during adolescence, respectively, indicating that the behavioral effects of dopamine depletion depend on the period of lesion development. Dopamine depletion during adulthood induces hyperalgesic response to mechanical, thermal, and/or chemical stimuli, whereas the effects of neonatal dopamine depletion on nociceptive response in adolescent rats are yet to be examined. The latter aspect was addressed in this study, and behavioral responses were examined using von-Frey, tail flick, and formalin tests. The formalin test revealed that rats with neonatal dopamine depletion exhibited a significant increase in nociceptive response during interphase (6-15min post formalin injection) and phase 2 (16-75min post formalin injection). This increase in nociceptive response to the formalin injection was not reversed by pretreatment with methamphetamine, which ameliorates motor hyperactivity observed in adolescent rats with neonatal 6-OHDA treatment. The von-Frey filament and tail flick tests failed to reveal significant differences in withdrawal thresholds between neonatal 6-OHDA-treated and vehicle-treated rats. The spinal neuronal response to the formalin injection into the rat hind paw was also examined through immunohistochemical analysis of c-Fos protein. Significantly increased numbers of c-Fos-immunoreactive cells were observed in laminae I-II and V-VI of the ipsilateral spinal cord to the site of the formalin injection in rats with neonatal dopamine depletion compared with vehicle-treated rats. These results suggest that the dopaminergic neural system plays a crucial role in the development of a neural network for tonic pain, including the spinal neural circuit for nociceptive transmission, and that the mechanism underlying hyperalgesia to tonic pain is not always consistent with that of

Unusual towering elevation of troponin I after ST-elevation myocardial infarction and intensive monitoring with echocardiography post-percutaneous coronary intervention: a case report

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Suryadevara Ramya

2010-05-01

Full Text Available Abstract Introduction The elevation of troponin levels directly corresponds to the extent of myocardial injury. Here we present a case of a robust rise in cardiac biomarkers that correspond to extensive damage to the myocardium but did not spell doom for our patient. It is important to note that, to the best of our knowledge, this is the highest level of troponin I ever reported in the literature after a myocardial injury in an acute setting. Case presentation A 53-year-old African American man with an unknown medical history presented to the emergency room of our hospital with chest pain associated with diaphoresis and altered mental status. He required emergency intubation due to acute respiratory failure and circulatory collapse within 10 minutes of his arrival. He was started on heparin and eptifibatide (Integrilin drips but he was taken immediately for cardiac catheterization, which showed a total occlusion of his proximal left anterior descending, diffuse left circumflex disease and severe left ventricular dysfunction with segmental wall motion abnormality. He remained hypotensive throughout the procedure and an intra-aortic balloon pump was inserted for circulatory support. His urinary toxicology examination result was positive for cocaine metabolites. Serial echocardiograms showed an akinetic apex, a severely hypokinetic septum, and severe systolic dysfunction of his left ventricle. Our patient stayed at the Coronary Care Unit for a total of 15 days before he was finally discharged. Conclusion Studies demonstrate that an increase of 1 ng/ml in the cardiac troponin I level is associated with a significant increase in the risk ratio for death. The elevation of troponin I to 515 ng/ml in our patient is an unusual robust presentation which may reflect a composite of myocyte necrosis and reperfusion but without short-term mortality. Nevertheless, prolonged close monitoring is required for better outcome. We also emphasize the need for the

A review of endoparasitic acarines of Malaysia with special reference to novel endoparasitism of mites in amphibious sea snakes and supplementary notes on ecology of chiggers.

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Nadchatram, M

2006-06-01

Some 2,000 species of mites of the family Trombiculidae are known in the world. The 6-legged larvae are mostly ectoparasites of reptiles, birds, mammals and invertebrates. Their 8-legged active nymphs and adults are free-living predators. In the Asia-Pacific region, a few species in various genera are vectors of scrub typhus and scrub-itch. In this a paper, a very bizarre trombiculid species, Vatacarus ipoides Southcott 1957, endoparasitic in the trachea of the amphibious sea snake, Laticauda colubrina (Schenider) is re-described based mostly on new-born larvae reared in the laboratory. Life history study of the mite produced very novel and interesting results. A brief account of the life-cycle was presented at the first laboratory demonstration of the Malaysian Society of Parasitology and Tropical Medicine Meeting by Nadchatram and Audy (1965). The life history is illustrated and described here in greater detail. The active nymphal, and the akinetic teleiophane stages are bypassed, which is unusual in the life-cycle of the family Trombiculidae. Also, the larva is the only stage in the life-cycle that feeds. The sexes are predetermined in the larval neosomatic stage and give rise to small males and bigger females. Having obtained adults of the species, by rearing, it is deemed unnecessary for the original proposal by Southcott to erect a new family, Vatacaridae, because the adults share all the attributes of the family Trombiculidae. The male and female obtained through laboratory rearing are illustrated for the first time. Relationship of V. ipoides with Laticauda snakes show close host-specificity, in a group of acarines that are generally habitat-specific. Possible explanations for their association are discussed. The unusual morphology and the formation of new structures during an instar is of ontogenetic and evolutionary importance. The hypertrophic larvae are superficially vermiform, rather than typically acarine in shape. This, and other biological features

A Left Main Coronary Artery Thrombus Presenting as a Non St Elevation MI.

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Ezema, U; Daberkow, D; Delord, T; Guidry, L; Sells, N R

2017-01-01

Left main coronary artery (LMCA); thrombus with an acute myocardial infarction identified with coronary angiography is a clinically rare condition with an extremely high mortality rate. We present a case of LMCA thrombus that presented as a non-ST elevation myocardial infarction (NSTEMI);. A 45-year-old woman with a history of tobacco use and hyperlipidemia presented with a complaint of 10/10 "hard pain" across her chest radiating to her left shoulder and breast which woke her from sleep. The pain was constant and severe, with no alleviation with rest. She had not experienced anything like this before. Workup revealed an upward trending troponin (1.98.989.79);, and an EKG with some tachycardia but no ST elevation or T wave changes. Her CBC, CMP and coagulation studies were unremarkable. A toxicology screen was positive for opiates and benzodiazepines, medications she was on for pain and anxiety respectively. ACS protocol was started with DAPT, LMWH, Statin, ACEi, and Beta-blocker. An angiogram revealed a large thrombus in the LM coronary artery extending into the aorta with concomitant 99 percent stenosis of distal LAD. 2D Echo w/ bubble contrast was significant for PFO, akinetic apical inferior and anterior wall. The mid antero-septum and apical lateral wall were hypokinetic. Interventional Cardiology and CTS recommended conservative management with medical optimization (Continue DAPT, heparin);, watchful waiting for the thrombus to resorb. Left main coronary artery thrombosis (LMCAT); identified during coronary angiography is a rare and challenging condition. It is a life threatening condition with an approximate incidence rate of 0.8 percent . It is thought to be secondary to plaque rupture with subsequent thrombus formation that is associated with persistent hypercoagulable state, cocaine induced plaque rupture or coronary vasospasm, post-partum state and embolization of intra-cardiac masses. The patient presentation can vary from sudden cardiac death to

Could quantitative longitudinal peak systolic strain help in the detection of left ventricular wall motion abnormalities in our daily echocardiographic practice?

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Benyounes, Nadia; Lang, Sylvie; Gout, Olivier; Ancédy, Yann; Etienney, Arnaud; Cohen, Ariel

2016-10-01

Transthoracic echocardiography is the most commonly used tool for the detection of left ventricular wall motion (LVWM) abnormalities using "naked eye evaluation". This subjective and operator-dependent technique requires a high level of clinical training and experience. Two-dimensional speckle-tracking echocardiography (2D-STE), which is less operator-dependent, has been proposed for this purpose. However, the role of on-line segmental longitudinal peak systolic strain (LPSS) values in the prediction of LVWM has not been fully evaluated. To test segmental LPSS for predicting LVWM abnormalities in routine echocardiography laboratory practice. LVWM was evaluated by an experienced cardiologist, during routine practice, in 620 patients; segmental LPSS values were then calculated. In this work, reflecting real life, 99.6% of segments were successfully tracked. Mean (95% confidence interval [CI]) segmental LPSS values for normal basal (n=3409), mid (n=3468) and apical (n=3466) segments were -16.7% (-16.9% to -16.5%), -18.2% (-18.3% to -18.0%) and -21.1% (-21.3% to -20.9%), respectively. Mean (95% CI) segmental LPSS values for hypokinetic basal (n=114), mid (n=116) and apical (n=90) segments were -7.7% (-9.0% to -6.3%), -10.1% (-11.1% to -9.0%) and -9.3% (-10.5% to -8.1%), respectively. Mean (95% CI) segmental LPSS values for akinetic basal (n=128), mid (n=95) and apical (n=91) segments were -6.6% (-8.0% to -5.1%), -6.1% (-7.7% to -4.6%) and -4.2% (-5.4% to -3.0%), respectively. LPSS allowed the differentiation between normal and abnormal segments at basal, mid and apical levels. An LPSS value≥-12% detected abnormal segmental motion with a sensitivity of 78% for basal, 70% for mid and 82% for apical segments. Segmental LPSS values may help to differentiate between normal and abnormal left ventricular segments. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Extending Parent–Child Interaction Therapy for Early Childhood Internalizing Problems: New Advances for an Overlooked Population

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Puliafico, Anthony C.; Kurtz, Steven M. S.; Pincus, Donna B.; Comer, Jonathan S.

2014-01-01

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

In vivo signatures of nonfluent/agrammatic primary progressive aphasia caused by FTLD pathology

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Caso, Francesca; Mandelli, Maria Luisa; Henry, Maya; Gesierich, Benno; Bettcher, Brianne M.; Ogar, Jennifer; Filippi, Massimo; Comi, Giancarlo; Magnani, Giuseppe; Sidhu, Manu; Trojanowski, John Q.; Huang, Eric J.; Grinberg, Lea T.; Miller, Bruce L.; Dronkers, Nina; Seeley, William W.

2014-01-01

Objective: To identify early cognitive and neuroimaging features of sporadic nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA) caused by frontotemporal lobar degeneration (FTLD) subtypes. Methods: We prospectively collected clinical, neuroimaging, and neuropathologic data in 11 patients with sporadic nfvPPA with FTLD-tau (nfvPPA-tau, n = 9) or FTLD–transactive response DNA binding protein pathology of 43 kD type A (nfvPPA-TDP, n = 2). We analyzed patterns of cognitive and gray matter (GM) and white matter (WM) atrophy at presentation in the whole group and in each pathologic subtype separately. We also considered longitudinal clinical data. Results: At first evaluation, regardless of pathologic FTLD subtype, apraxia of speech (AOS) was the most common cognitive feature and atrophy involved the left posterior frontal lobe. Each pathologic subtype showed few distinctive features. At presentation, patients with nfvPPA-tau presented with mild to moderate AOS, mixed dysarthria with prominent hypokinetic features, clear agrammatism, and atrophy in the GM of the left posterior frontal regions and in left frontal WM. While speech and language deficits were prominent early, within 3 years of symptom onset, all patients with nfvPPA-tau developed significant extrapyramidal motor signs. At presentation, patients with nfvPPA-TDP had severe AOS, dysarthria with spastic features, mild agrammatism, and atrophy in left posterior frontal GM only. Selective mutism occurred early, when general neurologic examination only showed mild decrease in finger dexterity in the right hand. Conclusions: Clinical features in sporadic nfvPPA caused by FTLD subtypes relate to neurodegeneration of GM and WM in frontal motor speech and language networks. We propose that early WM atrophy in nfvPPA is suggestive of FTLD-tau pathology while early selective GM loss might be indicative of FTLD-TDP. PMID:24353332

Catatonia in 26 patients with bipolar disorder: clinical features and response to electroconvulsive therapy.

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Medda, Pierpaolo; Toni, Cristina; Luchini, Federica; Giorgi Mariani, Michela; Mauri, Mauro; Perugi, Giulio

2015-12-01

We describe the clinical characteristics and short-term outcomes of a sample of inpatients with bipolar disorder with severe catatonic features resistant to pharmacological treatment. The study involved 26 catatonic patients, resistant to a trial of benzodiazepines, and then treated with electroconvulsive therapy (ECT). All patients were evaluated prior to and one week following the ECT course using the Bush-Francis Catatonia Rating Scale (BFCRS) and the Clinical Global Impression (CGI). In our sample, women were over-represented (n = 23, 88.5%), the mean (± standard deviation) age was 49.5 ± 12.5 years, the mean age at onset was 28.1 ± 12.8 years, and the mean number of previous mood episodes was 5.3 ± 2.9. The mean duration of catatonic symptoms was 16.7 ± 11.8 (range: 3-50) weeks, and personal history of previous catatonic episodes was present in 10 patients (38.5%). Seventeen (65.4%) patients showed abnormalities at cerebral computerized tomography and/or magnetic resonance imaging and neurological comorbidities were observed in 15.4% of the sample. Stupor, rigidity, staring, negativism, withdrawal, and mutism were observed in more than 90% of patients. At the end of the ECT course, 21 patients (80.8%) were classified as responders. The BFCRS showed the largest percentage of improvement, with an 82% reduction of the initial score. The number of previous mood episodes was significantly lower and the use of anticholinergic and dopamine-agonist medications was significantly more frequent in non-responders than in responders. Our patients with bipolar disorder had predominantly retarded catatonia, frequent previous catatonic episodes, indicating a recurrent course, and high rates of concomitant brain structure alterations. However, ECT was a very effective treatment for catatonia in this patient group that was resistant to benzodiazepines. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

DTI fiber tractography of cerebro-cerebellar pathways and clinical evaluation of ataxia in childhood posterior fossa tumor survivors.

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

2017-01-01

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

World War I psychoneuroses: hysteria goes to war.

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Tatu, Laurent; Bogousslavsky, Julien

2014-01-01

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'.

Twelve clinically significant points in medulloblastoma

International Nuclear Information System (INIS)

Sugiyama, Kazuhiko; Yamasaki, Fumiyuki; Kajiwara, Yoshinori; Watanabe, Yosuke; Takayasu, Takeshi; Kurisu, Kaoru

2011-01-01

Though medulloblastoma is the most common malignant brain tumor during childhood, only 80 newly-diagnosed tumors develos every year, as the annual incidence is extremely rare with an occurrence of 0.5 per 100,000 children younger than 15-year-old and of 0.7 per 100,000 for the entire population. Images obtained of medulloblastoma are characterized by a round heterogeously-enhanced mass in or adjacent to the VIth ventricle. Objectives of surgical treatment are the maximum resectioning of the main mass and the relief of the obstructive hydrocephalus. Cerebellar mutism occurs a few days after one fourth of medulloblastoma surgery, and lasts approximately for 50 days followed by subsequent dysarthria. Pathological subtypes include classic medulloblastoma, desmoplastic/nodular medulloblastoma, medulloblastoma with extensive nodularity, large cell/anaplastic medulloblastoma, all corresponding to World Health Organization (WHO) grade IV. According to age, residual tumor size, and disseminated staging, patients are divided into average-risk group, high-risk group, or baby-medulloblastoma after surgery. Standard treatment in average-risk group includes 23.4-Gy cranio-spinal irradiation (CSI) with posterior boost followed by chemotherapy consisting of cisplatin (CDDP), alkylating agents, and vincristine. Patients in high-risk group receive over 36-Gy CSI with boost radiotherapy to nodular lesions before, concomitantly with, or followed by dose-intensity chemotherapy. In cases with gross total removal, or desmoplastic/nodular pathology radiotherapy for patients younger than 3-year-old are often delayed until they turn 3-year-old, and are able to survive for long time by appropriate chemotherapy alone. Adolescent survivors with childhood medulloblastoma have a number of late adverse effects regarding another neoplasm, neuro-cognitive function, endocrine activity, cardiovascular organs, and skeletal system. Comprehensive follow-up and support system are mandatory. (author)

Extending parent-child interaction therapy for early childhood internalizing problems: new advances for an overlooked population.

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

2014-12-01

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

Comparison of Nitrate-augmented resting gated 99mTc-Sestamibi imaging with Low Dose Dobutamine SPECT for the detection of Myocardial Viability

International Nuclear Information System (INIS)

Parameswaran, R.V.; Dash, P.K.; Barooah, B.; Guruprasad, H.P.; Purantharan, N.

2002-01-01

Background: Dobutamine Echocardiography and Radionuclide imaging with Tl-201 and Technetium agents are two of the most established techniques available for the detection of viable myocardium. The purpose of this study was to evaluate the utility of Low dose Dobutamine gated Myocardial SPECT in identifying additional areas of dysfunctional, but viable myocardium when compared to nitrate-augmented rest gated SPECT with SestaMIBI. Materials and Methods: 20 patients (19 males and 1 female, with an age range of 40- 65 yrs and a mean of 52.75yrs) all with history of MI or severe LV dysfunction were included in this study. Patients with LBBB, recent revascularisation, arrhythmias etc were excluded from the study. A routine stress-rest study was initially performed on them with 99mTc-SestaMIBI and both the stress as well as resting studies was gated and the resting study was augmented with 10mg of sublingual nitrate. After the resting study was over, the patient was infused with Low dose Dobutamine(5μg/kg/min), at which time the gated acquisition was started and the infusion was continued till the acquisition got completed. In the perfusion study with SestaMIBI, all infarct segments which have uptake less than 50% as compared to the maximally perfused area was deemed non-viable Results: A 17-segment Myocardial model was used for both perfusion as well as quantification of wall motion and wall thickening. 110 akinetic/dyskinetic segments were taken up for analysis. 93 of these segments were non-viable and improvement in wall motion was seen in 17 segments (15.4%) in the nitrate-augmented SPECT. Dobutamine study showed improvement in totally in 27 segments (24.5%), 10 of which were additional segments which were non-viable in Nitrate SPECT study, apart from the 17 segments which showed improvement in the Nitrate study. In Dobutamine study, there was also improvement in wall thickening in 10 (10.7%) out of 93 segments which had showed less than 50% of MIBI uptake. Conclusion

The significance of resting thallium-201 delayed SPECT for assessing viability of infarcted regions

International Nuclear Information System (INIS)

Mori, Takao; Yamabe, Hiroshi; Yoshida, Hiroyuki; Maeda, Kazumi; Fukuzaki, Hisashi

1991-01-01

Thirty-eight patients with old myocardial infarction (OMI) and 35 patients with effort angina pectoris (EAP) underwent resting thallium-201 (Tl) SPECT one week after exercise (Ex) Tl SPECT. On the basis of both resting and Ex Tl SPECT scans, OMI patients were divided into 3 groups: those who had Tl redistribution (RD) on Ex images and the same perfusion defect (PD) on both resting and Ex delayed images (n=12, Group 1); those who had a lower PD on resting delayed images than Ex delayed images (n=15, Group 2); and those who had no Tl RD on Ex images in spite of the same PD on both resting and EX delayed images (n=11, Group 3). EAP patients were divided into Group 1 (n=18) in whom no PD was seen on Ex delayed image and Group 2 (n=17) in whom PD was not seen on Ex delayed images either, but seen on resting delayed images. EX images showed higher pulmonary artery wedge pressure in Group 2 than Group 1 of both OMI and EAP patients (27±7 mmHg vs 15±6 mmHg for OMI patients; 22±6 mmHg vs 12±7 mmHg for EAP patients). For PD, Tl uptake rate on Ex initial images was higher in Group 1 than Gorup 2 of both OMI and EAP patients (63±7% vs 55±9% for OMI patients; 72±7% vs 65±9% for EAP patients). Resting delayed images for OMI patients showed the highest Tl uptake rate in Group 1 (78±7%), followed by Group 2 (74±8%) and then Group 3 (41±10%). In the group of OMI patients, the incidence of akinetic or dyskinetic wall motion on left ventriculography was the highest in Group 3 (85.7%), followed by Group 2 (52.2%) and Group 1 (24.3%). OMI patients, as well as EAP patients, had lower PD on resting delayed images than EX delayed images. Thus, severer ischemia may be associated with stress and infarcted areas may not involve viable muscle when Tl PD is decreased on resting delayed images. (N.K.)

Neurological Effects of Acute Carbon Monoxide Poisoning in Children

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Coskun YARAR

2009-11-01

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

Non-Motor Symptoms of Parkinson’s Disease and Their Impact on Quality of Life in a Cohort of Moroccan Patients

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Houyam Tibar

2018-04-01

Full Text Available BackgroundNon-motor symptoms (NMSs are a real burden in Parkinson’s disease (PD. They may appear in early pre-symptomatic stage as well as throughout the disease course. However, their relationship with the deterioration of the patient’s quality of life (QoL is still under debate. This study aimed to investigate the prevalence of NMSs and their impact on the QoL in a cohort of Moroccan patients.MethodsWe carried out a cross-transactional study, where a total of 117 patients were submitted to a structured clinical interview and examination investigating motor and NMSs based on common and conventional scales. Motor symptoms were assessed by the UPDRS I–VI during ON condition. The NMSs were evaluated with common scales and their relationship with the QoL was investigated.ResultsThe mean patient’s age was 60.77 ± 11.36 years old, and the median disease duration was 6 years [2.5–9.5]. Motor’s phenotype subtypes were the mixed form in 40.2% of patients, akinetic-rigid in 20.5% and a tremor-dominant form in 39.3%. The median Hoehn and Yahr staging was 2 [1–2.5]. Regarding NMSs, the most common were urinary dysfunctions (82.6%, sleep (80.6%, and gastrointestinal (80% disorders. Other autonomic dysfunctions were also frequent: thermoregulatory dysfunctions 58.6%, cardiovascular troubles 50.9%, and sexual dysfunctions 47.9%. Depression was present in 47.9% and fatigue symptoms in 23.1%. The median score of SCOPA-AUT was 14 [7.75–21.80]. The median PD questionnaire 39-score index (PDQ39-SI was 23.22% and the most affected dimension was “mobility.” Univariate and multivariate analyses showed that the SCOPA-AUT score impacted the QoL (p = 0.001, especially the gastrointestinal (p = 0.007, and cardiovascular (p = 0.049 dimensions.ConclusionOur data show that all patients have presented at least one NMS. Autonomic and sleep disorders were the most frequent, and in contrast to other studies, digestive and cardiovascular

IMPULSIVE-COMPULSIVE DISORDERS IN PARKINSON'S DISEASE. CLINICAL CASES

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N. V. Fedorova

2015-01-01

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

Dobutamine stress magnetic resonance imaging suffices for the demonstration of myocardial ischaemia and viability.

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Lamers, F P L; van Dijkman, P R M; Kuijpers, Th J A; van Herpen, G

2003-02-01

We report three patients in whom dobutamine stress magnetic imaging (DS-MRI) was essential in assessing myocardial ischaemia. Two patients were referred to the cardiologist because of chest pain. Patient A had typical exertional angina and a normal resting electrocardiogram (ECG). Patient B had typical exercise-induced angina and had recently experienced an attack of severe chest pain at rest for 15 minutes. The ECG showed a complete left bundle branch block (LBBB). Patient C was referred for heart failure of unknown origin. There were no symptoms of chest pain during rest or exercise. Echocardiography in this patient demonstrated global left ventricular (LV) dilatation, systolic dysfunction and a small dyskinetic segment in the inferior wall. In all these patients exercise stress testing had failed to demonstrate myocardial ischaemia. Patients A and C produced normal findings whereas in patient B the abnormal repolarisation due to pre-existent LBBB precluded a diagnosis of ischaemia. Breath-hold DS-MRI was performed to study LV wall motion and wall thickening at rest through increasing doses of dobutamine. A test was considered positive for myocardial ischaemia if wall motion abnormalities developed at high-dose levels of the drug (20 μg/kg/min or more with a maximum of 40 μg/kg/min) in previously normal vascular territories or worsened in a segment that was normal at baseline. Recovery of wall thickening in a previously hypokinetic or akinetic segment at a low dose of dobutamine (5-10 μg/kg/min) was taken as proof of viability. Patients A and B developed hypokinesia progressing into akinesia at high-dose dobutamine in the anteroseptal area of the LV indicative of ischaemia. These findings were corroborated by coronary angiography demonstrating severe coronary artery disease which led to coronary artery bypass grafting (CABG) in patient A and balloon angioplasty in patient B. In patient C global recovery of LV contractions during low-dose dobutamine was

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

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Ojo Oluwadamilola O

2010-01-01

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

Assessment of regional left ventricular function by Dual Source Computed Tomography: Interobserver variability and validation to laevocardiography

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Pflederer, T. [Department of Internal Medicine 2 (Cardiology), University of Erlangen (Germany)], E-mail: tobiaspflederer@web.de; Ho, K.T. [Department of Cardiology, Tan Tock Seng Hospital (Singapore)], E-mail: contact@ttsh.com.sg; Anger, T. [Department of Internal Medicine 2 (Cardiology), University of Erlangen (Germany)], E-mail: thomas.anger@uk-erlangen.de; Kraehner, R. [Department of Internal Medicine 2 (Cardiology), University of Erlangen (Germany)], E-mail: robert.kraehner@uk-erlangen.de; Ropers, D. [Department of Internal Medicine 2 (Cardiology), University of Erlangen (Germany)], E-mail: dieter.ropers@uk-erlangen.de; Muschiol, G. [Department of Internal Medicine 2 (Cardiology), University of Erlangen (Germany)], E-mail: gerd.muschiol@uk-erlangen.de; Renz, A. [Department of Internal Medicine 2 (Cardiology), University of Erlangen (Germany)], E-mail: alexandra.renz@uk-erlangen.de; Daniel, W.G. [Department of Internal Medicine 2 (Cardiology), University of Erlangen (Germany)], E-mail: werner.daniel@uk-erlangen.de; Achenbach, S. [Department of Internal Medicine 2 (Cardiology), University of Erlangen (Germany)], E-mail: stephan.achenbach@uk-erlangen.de

2009-10-15

Objective: Assessment of left ventricular function is possible in contrast-enhanced cardiac CT data sets. However, rapid ventricular motion especially in systole can lead to artifacts. Dual Source Computed Tomography (DSCT) has high temporal resolution which effectively limits motion artifact. We therefore assessed the accuracy of DSCT to detect regional left ventricular wall motion abnormalities in comparison to invasive cine angiocardiography. Methods: We analyzed DSCT data sets of 50 patients (39 male, 11 female, mean age: 61 {+-} 10 years) which were acquired after intravenous injection of 55-70 mL contrast agent (rotation time: 330 ms, collimation: 2 mm x 64 mm x 0.6 mm, 120 kV, 380 mAs, ECG-correlated tube current modulation). 10 data sets consisting of transaxial slices with a slice thickness of 1.5 mm, an increment of 1.0 mm and a matrix of 256 x 256 pixels were reconstructed at 10 time instants during the cardiac cycle (0-90% in 10% increments). The data sets were analyzed visually by two independent readers, using standard left ventricular planes, concerning regional wall motion abnormalities. DSCT was verified in a blinded fashion against cine ventriculography performed during cardiac catheterization (RAO and LAO projection), using a 7-segment model. Analysis was performed on a per-patient (presence of at least one hypo-, a- or dyskinetic segment) and on a per-segment basis. Results: Concerning the presence of a wall motion abnormality, the two observers agreed in 340/350 segments (97%) and 48/50 patients (96%). In invasive cine angiocardiography, 22 of 50 patients displayed at least one segment with abnormal contraction. To detect these patients, DSCT showed a sensitivity of 95% (21/22), specificity of 96% (27/28), positive predictive value of 95% and negative predictive value of 96%. Out of a total of 350 left ventricular segments, 66 segments had abnormal contraction in cine angiocardiography (34 hypokinetic, 26 akinetic, 6 dyskinetic). For detection

A Putative O-Linked β-N-Acetylglucosamine Transferase Is Essential for Hormogonium Development and Motility in the Filamentous Cyanobacterium Nostoc punctiforme.

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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

2017-05-01

the most developmentally complex prokaryotes. Species such as Nostoc punctiforme develop an array of cell types, 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. Copyright © 2017 American Society for Microbiology.

Mineralized remains of morphotypes of filamentous cyanobacteria in carbonaceous meteorites

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Hoover, Richard B.

2005-09-01

The quest for conclusive evidence of microfossils in meteorites has been elusive. Abiotic microstructures, mineral grains, and even coating artifacts may mimic unicellular bacteria, archaea and nanobacteria with simple spherical or rod morphologies (i.e., cocci, diplococci, bacilli, etc.). This is not the case for the larger and more complex microorganisms, colonies and microbial consortia and ecosystems. Microfossils of algae, cyanobacteria, and cyanobacterial and microbial mats have been recognized and described from many of the most ancient rocks on Earth. The filamentous cyanobacteria and sulphur-bacteria have very distinctive size ranges, complex and recognizable morphologies and visibly differentiated cellular microstructures. The taphonomic modes of fossilization and the life habits and processes of these microorganisms often 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). Valid biogenicity is provided by the combination of a suite of known biogenic elements (that differ from the meteorite matrix) found in direct association with recognizable and distinct biological features and microstructures (e.g., uniseriate or multiseriate filaments, trichomes, sheaths and cells of proper size/size range); specialized cells (e.g., basal or apical cells, hormogonia, akinetes, and heterocysts); and evidence of growth characteristics (e.g., spiral filaments, robust or thin sheaths, laminated sheaths, true or false branching of trichomes, tapered or uniform filaments) and evidence of locomotion (e.g. emergent cells and trichomes, coiling hormogonia, and hollow or flattened and twisted sheaths). Since 1997 we have conducted Environmental and Field Emission Scanning Electron Microscopy (ESEM and FESEM) studies of freshly fractured interior surfaces of carbonaceous meteorites, terrestrial

The evolution of primary progressive apraxia of speech.

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Josephs, Keith A; Duffy, Joseph R; Strand, Edythe A; Machulda, Mary M; Senjem, Matthew L; Gunter, Jeffrey L; Schwarz, Christopher G; Reid, Robert I; Spychalla, Anthony J; Lowe, Val J; Jack, Clifford R; Whitwell, Jennifer L

2014-10-01

Primary progressive apraxia of speech is a recently described neurodegenerative disorder in which patients present with an isolated apraxia of speech and show focal degeneration of superior premotor cortex. Little is known about how these individuals progress over time, making it difficult to provide prognostic estimates. Thirteen subjects with primary progressive apraxia of speech underwent two serial comprehensive clinical and neuroimaging evaluations 2.4 years apart [median age of onset = 67 years (range: 49-76), seven females]. All underwent detailed speech and language, neurological and neuropsychological assessments, and magnetic resonance imaging, diffusion tensor imaging and (18)F-fluorodeoxyglucose positron emission tomography at both baseline and follow-up. Rates of change of whole brain, ventricle, and midbrain volumes were calculated using the boundary-shift integral and atlas-based parcellation, and rates of regional grey matter atrophy were assessed using tensor-based morphometry. White matter tract degeneration was assessed on diffusion-tensor imaging at each time-point. Patterns of hypometabolism were assessed at the single subject-level. Neuroimaging findings were compared with a cohort of 20 age, gender, and scan-interval matched healthy controls. All subjects developed extrapyramidal signs. In eight subjects the apraxia of speech remained the predominant feature. In the other five there was a striking progression of symptoms that had evolved into a progressive supranuclear palsy-like syndrome; they showed a combination of severe parkinsonism, near mutism, dysphagia with choking, vertical supranuclear gaze palsy or slowing, balance difficulties with falls and urinary incontinence, and one was wheelchair bound. Rates of whole brain atrophy (1.5% per year; controls = 0.4% per year), ventricular expansion (8.0% per year; controls = 3.3% per year) and midbrain atrophy (1.5% per year; controls = 0.1% per year) were elevated (P ≤ 0.001) in all 13

[Epidemioclinical and legal aspects and cost management of sexual abuse among minors in Dakar, Senegal].

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Cisse, C T; Niang, M M; Sy, A K; Faye, E H O; Moreau, J-C

2015-11-01

Specify epidemioclinical and legal aspects of sexual abuse among minors and evaluate the cost of care in Dakar. This is a retrospective multicenter cross-sectional study on sexual abuse among minors over a period of four years from 1st January 2006 to 31st December 2009. Four maternities were targeted: the Social Hygiene Institute of Medina, health center Roi-Baudouin Guédiawaye, the Pikine hospital and health center Youssou-Mbargane-Diop of Rufisque. During the study period, 252 child victims of sexual abuse were supported at four health facilities on a total of 272 sexual abuses of all ages, a frequency of 92.64%. The epidemiological profile of our patients was a child of 11 years old on average, female (100%) and living in the suburbs of Dakar (68.1%). Children were often abused during working hours (31.7%), outside the family environment and often by someone known to the victim (72.6%). Genito-genital contact was the most common mode of sexual contact (80.9%) with vaginal penetration in 61% of cases. Almost all of the victims (92.1%) came to consult, accompanied by their parents, between the 1st and 4th day after the sexual abuse (70%). The examination usually revealed a hymenal trauma (59.9%) of which nearly half (49%) consisted of old lesions. 56.9% of victims had a post-traumatic stress disorder and 31.1%, mutism. We recorded six (6) pregnancies, 2% of our sample. A case of HIV infection was recorded on a sample taken 72hours after sexual abuse. Control of three months HIV serology was requested in 7.1% of cases and only 20% of children had received antiretroviral prophylaxis. Antibiotic prophylaxis had been performed in 13.7% of cases using doxycycline as drug of choice (75%). Only 29% of our patients had received emergency contraception progestin and psychological care concerned only 22% of children. On the legal aspects, 46% of our patients had filed a complaint. Prosecutions were 38%, 45% of which were convicted and 21% were acquitted. The rate of

Eficácia da fonoterapia em um caso de afasia expressiva decorrente de acidente vascular encefálico Speech therapy effectiveness in a case of expressive aphasia resulting from stroke

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Letícia Regina Kunst

2012-01-01

a male patient, aged 68, treated in the clinic since May 2009. His therapeutic process was analyzed up to December 2010. The client suffered an ischemic stroke in the left cerebral hemisphere, on January 2009, when suddenly presented mutism. After the evaluations, the therapist concluded that the client presented "aphasia, compromising the oral and the writing expression, caused by ischemic stroke". So, the treatment plan was created. Since the start of therapy, significant linguistic changes could be observed in the client. A primary factor for the satisfactory recover was the quick search for the speech treatment (15 days after the stroke, because there is a degree of spontaneous recovery in aphasic clients. But, the good results are minimal without the adequate therapy. Only one month after the beginning of the therapy, the client improved substantially his vocabulary. Despite the injury area is extensive and fundamental to the language, it was observed very positive results with the therapy. So, we believe that the early speech therapy contributed a lot to the linguistic recover, since the client communicate satisfactorily, if we consider the great extension of his injury.

Reconstrução fisiológica do ventrículo esquerdo: o conceito de máxima redução ventricular e mínima resposta inflamatória Physiologic left ventricular reconstruction: the concept of maximum ventricular reduction and minimum inflammatory reaction

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Walter J. Gomes

2004-12-01

induce a chronic myocardial inflammatory reaction. We report on a modification of the ventricular reconstruction technique that eliminates the need of intraventricular patches and the use of synthetic material. METHOD: Eleven consecutive patients presenting with left ventricular aneurysms, evolving to functional class III and IV of the New York Heart Association, underwent direct left ventricular reconstruction surgery without the use of intraventricular patches or prosthetic strips. RESULTS: There was no operative mortality or need of mechanical circulatory support. The postoperative hospital stay ranged from 4 to 7 days (average 5.3 ± 1.1 days. The serial echocardiogram control showed reduction of the left ventricular diastolic diameter (from 69.0± 7.5 mm preoperatively to 62.6 ± 5.1 mm postoperatively. The left ventricular ejection fraction increased from 47.3% ± 6.6% to 56.3% ±10.5%. One-year follow-up revealed eight patients in functional class I and three in class II. CONCLUSION: This technique, with elimination of prosthetic materials, could contribute to an improvement of the clinical results in patients who undergo left ventricular reconstruction, providing virtual elimination of left ventricular akinetic areas and potentially attenuating the long-term myocardial chronic inflammatory reaction.

Tratamento cirúrgico dos aneurismas de ventrículo esquerdo e isquemia coronária

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Danton R. da Rocha LOURES

1997-04-01

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.

[Catatonia: resurgence of a concept. A review of the international literature].

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Pommepuy, N; Januel, D

2002-01-01

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

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

1999-12-01

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

[Frontal dementia or dementia praecox? A case report of a psychotic disorder with a severe decline].

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Vanderzeypen, F; Bier, J C; Genevrois, C; Mendlewicz, J; Lotstra, F

2003-01-01

anatomical abnormality. Frontotemporal lobar degeneration (FTLD) is one of the most common causes of cortical dementia. FTLD is associated with an anatomical atrophy that can be generalised, with a frontotemporal or focal lobar predominance. Histologically there is severe neuronal loss, gliosis and a state of spongiosis. In a minority of case Pick cells and Pick bodies are also found. The usual clinical features of FTLD are divided in three prototypic syndromes: frontotemporal dementia (FTD), progressive non-fluent aphasia (PA) and semantic dementia (SD). FTD is the most common clinical manifestation of FTLD. FTD is first characterised by profound alteration in personality and social conduct, characterised by inertia and loss of volition or social disinhibition and distractibility. There is emotional blunting and loss of insight. Speech output is typically economical, leading ultimately to mutism, although a press of speech may be present in some overactive, disinhibited patients. Memory is relatively preserved in the early stage of the disease. Cognitive deficits occur in the domains of attention, planning and problems solving, whereas primary tools of language, perception and spatial functions are well preserved. PA is an initial disorder of expressive language, characterised by effortful speech production, phonologic and grammatical errors. Difficulties in reading and writing also occur but understanding of word meaning is relatively well preserved. In SD a severe naming and word comprehension impairment occur on the beginning in the context of fluent, effortless, and grammatical speech output. There is also an inability to recognise the meaning of visual percepts. The clinical syndromes of FTLD are associated with the brain topography of the degeneration. So considerable clinical overlap can exist between schizophrenia and FTLD and the object of the following case report is to remind the difficulty to make a differential diagnosis between these two pathologies. A 34 year

Stem Cell Therapy for Congestive Heart Failure

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Gunduz E

2011-01-01

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

Total allowable concentrations of monomeric inorganic aluminum and hydrated aluminum silicates in drinking water.

Science.gov (United States)

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

2012-05-01

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�