Rapid Response of Long-Standing, Treatment-Resistant Non-Catatonic Mutism in Paranoid Schizophrenia with Single ECT session.

PubMed

Dar, Mansoor Ahmad; Rather, Yasir Hassan; Shah, Majid Shafi; Wani, Rayees Ahmad; Hussain, Arshad

2014-11-01

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

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

ERIC Educational Resources Information Center

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…

The Development and Psychometric Properties of the Selective Mutism Questionnaire

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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…

Mutism as the Presenting Symptom: Three Case Reports and Selective Review of Literature

PubMed Central

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. PMID:21799563

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

PubMed

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.

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

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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,…

The Cerebellar Mutism Syndrome and Its Relation to Cerebellar Cognitive Function and the Cerebellar Cognitive Affective Disorder

ERIC Educational Resources Information Center

Wells, Elizabeth M.; Walsh, Karin S.; Khademian, Zarir P.; Keating, Robert F.; Packer, Roger J.

2008-01-01

The postoperative cerebellar mutism syndrome (CMS), consisting of diminished speech output, hypotonia, ataxia, and emotional lability, occurs after surgery in up to 25% of patients with medulloblastoma and occasionally after removal of other posterior fossa tumors. Although the mutism is transient, speech rarely normalizes and the syndrome is…

Cerebellar mutism--report of four cases.

PubMed

Ozimek, A; Richter, S; Hein-Kropp, C; Schoch, B; Gorissen, B; Kaiser, O; Gizewski, E; Ziegler, W; Timmann, D

2004-08-01

The aim of the present study was to investigate the manifestations of mutism after surgery in children with cerebellar tumors. Speech impairment following cerebellar mutism in children was investigated based on standardized acoustic speech parameters and perceptual criteria. Mutistic and non-mutistic children after cerebellar surgery as well as orthopedic controls were tested pre-and postoperatively. Speech impairment was compared with the localization of cerebellar lesions (i. e. affected lobules and nuclei). Whereas both control groups showed no abnormalities in speech and behavior, the mutistic group could be divided into children with dysarthria in post mutistic phase and children with mainly behavioral disturbances. In the mutistic children involvement of dentate and fastigial nuclei tended to be more frequent and extended than in the nonmutistic cerebellar children. Cerebellar mutism is a complex phenomenon of at least two types. Dysarthric symptoms during resolution of mutism support the anarthria hypothesis, while mainly behavioral changes suggest an explanation independent from speech motor control.

Transient mutism and pathologic laughter in the course of cerebellitis.

PubMed

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.

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

ERIC Educational Resources Information Center

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…

Postoperative cerebellar mutism and autistic spectrum disorder.

PubMed

Tasdemiroğlu, Erol; Kaya, Miktat; Yildirim, Can Hakan; Firat, Levent

2011-06-01

I read the article "An Inside View of Autism" written by a 44-year-old autistic woman who had a successful international career designing livestock equipment. In this article, she wrote about her life, disease, and experiences as an autistic individual. She stated that "It is interesting that my speech resembled the stressed speech in young children who have had tumors removed from the cerebellum". In this article, we intend to review and extensively document both postoperative cerebellar mutism and autistic spectrum disorder. We reviewed the clinical and neurological findings, etio-pathogenesis, neuroanatomy, mechanisms of development, and similarities between the etio-pathogenesis of both diseases. Cerebellar lesions can produce mutism and dysarthria, symptoms sometimes seen in autistic spectrum disorder. In mammals, cerebellar lesions disturb motivated behavior and reduce social interactions, functions that are disturbed in autistic spectrum disorder and cerebellar mutism. The cerebellum and two regions within the frontal lobes are active in certain language tasks. Language is abnormal in autistic spectrum disorder and cerebellar mutism.

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?

ERIC Educational Resources Information Center

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…

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

PubMed

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.

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

PubMed Central

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. PMID:29568498

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

PubMed

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.

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

PubMed

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.

Treatment resistant non-catatonic mutism in schizophrenia responding to a combination of continuation electroconvulsive therapy and neuroleptics

PubMed Central

Grover, Sandeep; Dutt, Alakananda; Chakrabarty, Kaustav; Kumar, Vineet

2012-01-01

Non-catatonic mutism in schizophrenia has been described less frequently in literature. We describe the case of a young male who presented with non-catatonic mutism, secondary to first rank symptoms, which was refractory to adequate antipsychotic trials (quetiapine, risperidone, aripiprazole, ziprasidone, and trifluperazine) and responded to a combination of electroconvulsive therapy (ECT) and neuroleptics partially. However, when the ECT was continued in the continuation phase, the patient started speaking. PMID:23766583

Spino-Cerebellar Degeneration, Hormonal Disorder, Hypogonadism, Deaf Mutism and Mental Deficiency

ERIC Educational Resources Information Center

Sylvester, P. E.

1972-01-01

Post mortem examinations were done on two adult siblings (one female and one male) who had been clinically described as suffering from mental handicap, deaf mutism, ataxia, hypogonadism, and hormonal disorders. (DB)

Mutism: elective or selective, and acquired.

PubMed

Gordon, N

2001-03-01

When a child does not speak, this may be because there is no wish to do so (elective or selective mutism), or the result of lesions in the brain, particularly in the posterior fossa. The characteristics of the former children are described, especially their shyness; and it is emphasized that mild forms are quite common and a definitive diagnosis should only be made if the condition is significantly affecting the child and family. In the case of mutism due to organic causes, the commonest of these is trauma to the cerebellum. Operations on the cerebellum to remove tumours can be followed by mutism, often after an interval of a few days, and it may last for several months or longer, to be followed by dysarthria. Other rarer causes are discussed, and also the differential diagnosis. The so-called posterior fossa syndrome consists of mutism combined with ataxia, cranial nerve palsies, bulbar palsies, hemiparesis, cognitive impairment and emotional lability, but the post-operative symptoms are often dominated by the lack of speech. The most accepted cause for the condition is vascular spasm with involvement of the dentate nucleus and the dentatorubrothalamic tracts to the brain-stem, and subsequently to the cortex. Diaschisis may be involved in causing the loss of higher cerebral functions, and possibly, complicating hydrocephalus. The treatment of elective mutism is reviewed, either using a psychotherapeutic approach or a variety of drugs, or both. These may well be ineffective, and it must be remembered that the condition often resolves on its own. The former treatment must concentrate on the training of social skills and activities of daily life and must be targeted to both the child, the family, and the school. Also, all kinds of punishment and insistence on speech must be discouraged. The drug, which seems to be most effective, is fluoxetine. Discovering more about the causes of mutism due to organic causes may well depend on studies using such techniques as magnetic resonance imaging and single photon emission tomography.

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

ERIC Educational Resources Information Center

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.

Treating selective mutism using modular CBT for child anxiety: a case study.

PubMed

Reuther, Erin T; Davis, Thompson E; 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 established treatment for anxiety--Modular Cognitive-Behavioral Therapy for Childhood Anxiety Disorders (Chorpita, 2007). The treatment consisted of 21 sessions and included modules on psychoeducation, exposure, cognitive restructuring, social skills, and maintenance and relapse prevention. The client's symptoms were greatly improved by the end of treatment based on fear hierarchy ratings, self-report and parent-report questionnaires, and child and parent clinical interviews. In addition, at discharge the client no longer met criteria for selective mutism. Improvements were maintained when the client was reassessed at 1-month and 6-month follow-up appointments.

Phenomenology and treatment of selective mutism.

PubMed

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.

[Rare consciousness disturbances in toxicological practice: akinetic mutism, somnambulism, locked-in syndrome, and psychogenic coma].

PubMed

Ciszowski, Krzysztof; Mietka-Ciszowska, Aneta

2013-01-01

The toxicity of xenobiotics can result inrare disorders of consciousness, such as akinetic mutism and somnambulism as well as syndromes mimicking consciousness disturbances, such as locked-in syndrome and psychogenic coma. Akinetic mutism is a condition characterized by a lack of spontaneous movements and little or no vocalization. Somnambulism include performing of complex motor activity in an automatic manner during deep sleep, without any awareness of its execution. The locked-in syndrome is a state with quadriplegia coexisting with cranial nerves palsies and mutism, but with fully preserved consciousness. Psychogenic coma is a condition in which the patient has preserved level of consciousness and awareness, but does not communicate with theenvironment and does not exhibit the external manifestations of consciousness. This paper presents the etiology, clinical characteristics, as well as diagnostic and therapeutic issues for the above syndromes.

Selective Mutism: Causes and Interventions.

ERIC Educational Resources Information Center

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)

Elective Mutism Associated with Selective Inactivity.

ERIC Educational Resources Information Center

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)

[Treatment of selective mutism].

PubMed

Melfsen, Siebke; Warnke, Andreas

2007-11-01

Selective mutism is a communication disorder of childhood in which the child does not speak in specific social situations despite the ability to speak in other situations. A literature review was completed in order to provide practical guidelines for the assessment and treatment of children with selective mutism. There are many different behavioral approaches in the treatment of this disorder, e.g. contingency management, shaping, stimulus fading, escape-avoidance, self-modeling, learning theory approaches. A clearer diagnostic understanding of the disorder as part of anxiety or oppositional disorders needs to be realized prior to generalize an effective treatment for this disorder.

Switching to aripiprazole for the treatment of residual mutism resulted in distinct clinical courses in two catatonic schizophrenia cases.

PubMed

Muneoka, Katsumasa; Kanahara, Nobuhisa; Kimura, Shou

2017-01-01

The efficacy of a partial agonist for the dopamine D 2 receptor, aripiprazole, for catatonia in schizophrenia has been reported. We report distinct clinical courses in challenging aripiprazole to treat residual mutism after severe catatonic symptoms improved. In the first case, mutism was successfully treated when the patient was switched from olanzapine to aripiprazole. In contract, switching to aripiprazole from risperidone aggravated auditory hallucinations in the second case. We will discuss the benefits and risks of using aripiprazole for the treatment of catatonic schizophrenia and the possibility of dopamine supersensitivity psychosis.

Silent Suffering: Children with Selective Mutism

ERIC Educational Resources Information Center

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…

Selective Mutism: Definition, Issues, and Treatment.

ERIC Educational Resources Information Center

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…

Functional Assessment-Based Intervention for Selective Mutism

ERIC Educational Resources Information Center

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…

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

PubMed

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.

[Selective mutism].

PubMed

Lämsä, Teemu; Erkolahti, Ritva

2013-01-01

Selective mutism is a disorder closely linked to anxiety disorders in particular social phobia. Heightened sensitivity to emotional stress factors and coping with social anxiety may be part of the causes leading to the failure to speak. Follow-up studies confirm the fact that selective mutism is a very persistent disorder with a general tendency of poor outcome in particular if not intensively treated. Current treatment combines different modalities of cognitive-behavioural therapies which are aimed to reduce anxiety symptoms and to improve verbal communication. Enhancing of parent-child interaction is often needed to improve the child's emotion regulation skills. SSRI medication, especially fluoxetine, may improve outcome when combined with therapy.

Multimethod Behavioral Treatment of Long-Term Selective Mutism.

ERIC Educational Resources Information Center

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: Phenomenological Characteristics.

ERIC Educational Resources Information Center

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 in Elementary School: Multidisciplinary Interventions.

ERIC Educational Resources Information Center

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.…

Selective Mutism: Treating the Silent Child

ERIC Educational Resources Information Center

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…

Augmented Self-Modeling as an Intervention for Selective Mutism

ERIC Educational Resources Information Center

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…

Narrative Skills in Children with Selective Mutism: An Exploratory Study

ERIC Educational Resources Information Center

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…

Increasing Verbal Behavior of a Student Who Is Selectively Mute

ERIC Educational Resources Information Center

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…

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

PubMed

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.

P50 Suppression in Children with Selective Mutism: A Preliminary Report

ERIC Educational Resources Information Center

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…

Differential Diagnosis of Selective Mutism in Bilingual Children

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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,…

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

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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…

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

PubMed

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.

Mutism and amnesia following high-voltage electrical injury: psychogenic symptomatology triggered by organic dysfunction?

PubMed

Mishra, Nishant K; Russmann, Heike; Granziera, Cristina; Maeder, Philippe; Annoni, Jean-Marie

2011-01-01

Mutism and dense retrograde amnesia are found both in organic and dissociative contexts. Moreover, dissociative symptoms may be modulated by right prefrontal activity. A single case, M.R., developed left hemiparesis, mutism and retrograde amnesia after a high-voltage electric shock without evidence of lasting brain lesions. M.R. suddenly recovered from his mutism following a mild brain trauma 2 years later. M.R.'s neuropsychological pattern and anatomoclinical correlations were studied through (i) language and memory assessment to characterize his deficits, (ii) functional neuroimaging during a standard language paradigm, and (iii) assessment of frontal and left insular connectivity through diffusion tractography imaging and transcranial magnetic stimulation. A control evaluation was repeated after recovery. M.R. recovered from the left hemiparesis within 90 days of the accident, which indicated a transient right brain impairment. One year later, neurobehavioral, language and memory evaluations strongly suggested a dissociative component in the mutism and retrograde amnesia. Investigations (including MRI, fMRI, diffusion tensor imaging, EEG and r-TMS) were normal. Twenty-seven months after the electrical injury, M.R. had a very mild head injury which was followed by a rapid recovery of speech. However, the retrograde amnesia persisted. This case indicates an interaction of both organic and dissociative mechanisms in order to explain the patient's symptoms. The study also illustrates dissociation in the time course of the two different dissociative symptoms in the same patient. Copyright © 2011 S. Karger AG, Basel.

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

ERIC Educational Resources Information Center

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,…

Selective Mutism: Practice and Intervention Strategies for Children

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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…

Language and Academic Abilities in Children with Selective Mutism

ERIC Educational Resources Information Center

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…

A Long-Term Outcome Study of Selective Mutism in Childhood

ERIC Educational Resources Information Center

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: Recommendations and a Case Study.

ERIC Educational Resources Information Center

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)

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

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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…

Assessment and Treatment of Selective Mutism with English Language Learners

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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.…

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

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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…

A controlled single-case treatment of severe long-term selective mutism in a child with mental retardation.

PubMed

Facon, Bruno; Sahiri, Safia; Rivière, Vinca

2008-12-01

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

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

PubMed

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.

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

PubMed Central

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

2015-01-01

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. PMID:26508972

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

ERIC Educational Resources Information Center

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),…

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

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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…

[Selective mutism].

PubMed

Rogoll, J; Petzold, M; Ströhle, A

2018-05-01

Selective mutism was first described in the medical literature 140 years ago. The diagnosis came into the focus of adult psychiatry with the appearance of DSM-5. Henceforth, selective mutism during infancy, adolescence and also adulthood is specified as an independent anxiety disorder. It often begins in early childhood with a kind of speechlessness in certain situations. A diagnostic clarification often only takes place after school enrolment. Very often comorbid anxiety disorders, especially social phobia and depression also occur. The course is very variable and with some affected persons regression of the pathology occurs suddenly and completely and with others there is a slow regression of the symptoms. Equally the disorder can persist until adulthood. Whilst formerly a traumatic genesis was assumed, a multifactorial etiology with genetic, psychological and language-associated effects is nowadays presumed. The therapy is supported through psychotherapy, speech therapy and psychopharmacology.

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

PubMed

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.

The development and psychometric properties of the selective mutism questionnaire.

PubMed

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

2008-04-01

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 speak related to SM. Study 1 (N = 589) utilized an Internet sample of parents of children ages 3 to 11 to demonstrate that the SMQ has a theoretically and clinically meaningful factor structure accounting for a significant portion of variance in responses with good internal consistency. Study 2 (N = 66) supported the validity of the SMQ in that scores discriminated clinic-referred children with SM from children with other anxiety disorders. Scores on the SMQ were correlated with measures of several theoretically and clinically important dimensions.

Selective mutism: an update and suggestions for future research.

PubMed

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.

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

PubMed

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.

Anxiety and oppositional behavior profiles among youth with selective mutism.

PubMed

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.

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

ERIC Educational Resources Information Center

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…

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

PubMed

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.

Treating youths with selective mutism with an alternating design of exposure-based practice and contingency management.

PubMed

Vecchio, Jennifer; Kearney, Christopher A

2009-12-01

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 functioning were employed, but particular focus was made on behavioral assessment of words spoken audibly and daily in public situations. Treatment ranged from 8 to 32 sessions and resulted in positive end-state functioning for 8 of 9 participants. Broader analyses indicated greater effectiveness for exposure-based practice than contingency management. The results support recent case reports of behavioral treatment for this population but in more rigorous fashion. Clinical and research challenges are discussed, including caveats about length and intensity of treatment for this population and need to develop standardized daily measures.

The Selective Mutism Questionnaire: Measurement Structure and Validity

PubMed Central

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

Pediatric selective mutism therapy: a randomized controlled trial.

PubMed

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 (P<0.001), Withdrawn (P=0.007) and Internalizing problems (P=0.020). Regarding SM severity according to SMQ assessment, children of group A showed a reduction of SM symptoms in all situations (school, P=0.003; family, P=0.018; and social, P=0.030 situations) and in SMQ total score (P<0.001). Our preliminary results suggest the positive effect of the psychomotor treatment in rehabilitative program for children affected by selective mutism, even if further studies are needed. The present study identifies in psychomotricity a safe and efficacy therapy for pediatric selective mutism.

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

PubMed

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.

BEHAVIORAL INTERVENTION TO TREAT SELECTIVE MUTISM ACROSS MULTIPLE SOCIAL SITUATIONS AND COMMUNITY SETTINGS

PubMed Central

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 with new children) and in three community settings. Results demonstrated increases in spoken initiations and responses and decreases in communication breakdowns across all situations and settings. PMID:21941394

Behavioral intervention to treat selective mutism across multiple social situations and community settings.

PubMed

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 with new children) and in three community settings. Results demonstrated increases in spoken initiations and responses and decreases in communication breakdowns across all situations and settings.

Differential Diagnosis of Selective Mutism in Bilingual Children

PubMed Central

TOPPELBERG, CLAUDIO O.; TABORS, PATTON; COGGINS, ALISSA; LUM, KIRK; BURGER, CLAUDIA

2012-01-01

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 “silent period”), specific diagnostic boundaries are not clear. The specific focus of this article is, therefore, the exact circumstances in which a language minority child should be diagnosed with SM. PMID:15908842

Silent suffering: understanding and treating children with selective mutism.

PubMed

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.

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

PubMed

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.

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

PubMed

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.

Elective Mutism: A Case Study of a Disorder of Childhood

PubMed Central

Louden, Delroy M.

1987-01-01

This paper reviews a number of treatment strategies utilized in the treatment of a 6-year-old boy with a history of multiple parental separations. When the patient presented to the child psychiatric clinic, he had a history of not speaking in the preceding four months, following the arrival of a stepmother in the household and a move to a new house in a new locality. Developmental and psychometric assessments are presented and their utility in early diagnosis and later prognosis are discussed. Reluctant speech is differentiated from, and compared with, elective mutism. The management of elective mutism is often complicated by these children not speaking to anyone in certain environments, and very frequently the persons with whom they do speak are not available for participation in treatment programs. Consequently, if a child does not speak to anyone who can participate in the early mapping of treatment modalities, the use of treatment strategies such as stimulus fading is impossible. Suggestions for future management to overcome this difficulty are presented and discussed. PMID:3316671

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

PubMed

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.

Cerebellar mutism syndrome and its relation to cerebellar cognitive and affective function: Review of the literature

PubMed Central

Yildiz, Ozlem; Kabatas, Serdar; Yilmaz, Cem; Altinors, Nur; Agaoglu, Belma

2010-01-01

Tumors of the cerebellum and brainstem account for half of all brain tumors in children. The realization that cerebellar lesions produce clinically relevant intellectual disability makes it important to determine whether neuropsychological abnormalities occur in long-term survivors of pediatric cerebellar tumors. Little is known about the neurobehavioral sequale resulting specifically from the resection of these tumors in this population. We therefore reviewed neuropsychological findings associated with postoperative cerebellar mutism syndrome and discuss the further implications for cerebellar cognitive function. PMID:20436742

Social phobia and selective mutism.

PubMed

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.

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

PubMed

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.

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

PubMed

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.

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

PubMed

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.

Post-operative paediatric cerebellar mutism syndrome: time to move beyond structural MRI.

PubMed

Toescu, Sebastian M; Hettige, Samantha; Phipps, Kim; Smith, R J Paul; Haffenden, Verity; Clark, Chris; Hayward, Richard; Mankad, Kshitij; Aquilina, Kristian

2018-06-20

To determine the value of structural magnetic resonance imaging (MRI) in predicting post-operative paediatric cerebellar mutism syndrome (pCMS) in children undergoing surgical treatment for medulloblastoma. Retrospective cohort study design. Electronic/paper case note review of all children with medulloblastoma presenting to Great Ormond Street Hospital between 2003 and 2013. The diagnosis of pCMS was established through a scoring system incorporating mutism, ataxia, behavioural disturbance and cranial nerve deficits. MRI scans performed at three time points were assessed by neuroradiologists blinded to the diagnosis of pCMS. Of 56 children included, 12 (21.4%) developed pCMS as judged by a core symptom of mutism. pCMS was more common in those aged 5 or younger. There was no statistically significant difference in pre-operative distortion or signal change of the dentate or red nuclei or superior cerebellar peduncles (SCPs) between those who did and did not develop pCMS. In both early (median 5 days) and late (median 31 months) post-operative scans, T2-weighted signal change in SCPs was more common in the pCMS group (p = 0.040 and 0.046 respectively). Late scans also showed statistically significant signal change in the dentate nuclei (p = 0.024). The development of pCMS could not be linked to any observable changes on pre-operative structural MRI scans. Post-operative T2-weighted signal change in the SCPs and dentate nuclei underlines the role of cerebellar efferent injury in pCMS. Further research using advanced quantitative MRI sequences is warranted given the inability of conventional pre-surgical MRI to predict pCMS.

Unable to Speak: Selective Mutism in Youth.

PubMed

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.

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

PubMed

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.

Reduced auditory efferent activity in childhood selective mutism.

PubMed

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.

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

PubMed

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.

Examination of a social problem-solving intervention to treat selective mutism.

PubMed

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

2008-03-01

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 discriminate salient social cues, select an appropriate social response, perform the response, and evaluate her performance. The girls generalized the skills to their respective regular classrooms and maintained the skills for up to 3 months after the removal of the intervention. Experimental control was demonstrated using a multiple baseline design across participants. Limitations of this study and issues for future research are discussed.

Selective Mutism Questionnaire: measurement structure and validity.

PubMed

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

2008-10-01

To evaluate the factor structure, reliability, and validity of the 17-item Selective Mutism Questionnaire (SMQ). 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; SM severity was assessed using the 17-item SMQ; and behavioral and affective symptoms were assessed using the Child Behavior Checklist. An exploratory factor analysis was conducted to investigate the dimensionality of the SMQ and a modified parallel analysis procedure was used to confirm exploratory factor analysis results. Internal consistency, construct validity, and incremental validity were also examined. The exploratory factor analysis yielded a 13-item solution consisting of three factors: social situations outside of school, school situations, and home and family situations. Internal consistency of SMQ factors and total scale ranged from moderate to high. Convergent and incremental validity was also well supported. Measure structure findings are consistent with the three-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 phenomena beyond other child anxiety measures.

Mumps encephalitis with akinesia and mutism.

PubMed

Suga, Kenichi; Goji, Aya; Shono, Miki; Matsuura, Sato; Inoue, Miki; Toda, Eiko; Miyazaki, Tatsushi; Kawahito, Masami; Mori, Kazuhiro

2015-08-01

Measles-rubella-mumps vaccination is routine in many countries, but the mumps vaccine remains voluntary and is not covered by insurance in Japan. A 5-year-old Japanese boy who had not received the mumps vaccine was affected by mumps parotitis. Several days later, he presented with various neurological abnormalities, including akinesia, mutism, dysphagia, and uncontrolled respiratory disorder. Mumps encephalitis was diagnosed. Despite steroid pulse and immunoglobulin treatment, the disease progressed. Magnetic resonance imaging showed necrotic changes in bilateral basal ganglia, midbrain, and hypothalamus. At 1 year follow up, he was bedridden and required enteral feeding through a gastric fistula and tracheostomy. Mumps vaccination should be made routine as soon as possible in Japan, because mumps encephalitis carries the risk of severe sequelae. © 2015 Japan Pediatric Society.

Refining the classification of children with selective mutism: a latent profile analysis.

PubMed

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

2008-10-01

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 an anxious-mildly oppositional group, an anxious-communication delayed group, and an exclusively anxious group. Follow-up tests indicated significant group differences on measures of SM symptom severity, externalizing problems, and expressive/receptive language abilities. These results suggest that, although social anxiety is typically a prominent feature of SM, children with the disorder are also likely to present with communication delays and/or mild behavior problems.

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

PubMed

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.

Narrative skills in children with selective mutism: an exploratory study.

PubMed

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

2004-11-01

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 abilities in 7 children with SM and a comparison group of 7 children with social phobia (SP). The children with SM produced significantly shorter narratives than children with SP, despite showing normal nonverbal cognitive and receptive language abilities. The findings suggest that SM may involve subtle expressive language deficits that may influence academic performance and raise additional questions for further research. The assessment procedure developed for this study may be potentially useful for language clinicians.

Children with autism spectrum disorders and selective mutism.

PubMed

Steffenburg, Hanna; Steffenburg, Suzanne; Gillberg, Christopher; Billstedt, Eva

2018-01-01

It has been suggested that autism spectrum disorder (ASD) might be a "comorbid" condition in selective mutism (SM). In this retrospective study, we examined medical records of children with SM diagnosis (n=97) at a medical center specializing in assessment of ASD. 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. The results highlight the risk of overlap between ASD and SM.

Selective mutism

MedlinePlus

... Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics . 20th ed. Philadelphia, ... Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics . 20th ed. Philadelphia, ...

Children with autism spectrum disorders and selective mutism

PubMed Central

Steffenburg, Hanna; Steffenburg, Suzanne; Gillberg, Christopher; Billstedt, Eva

2018-01-01

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. PMID:29765220

Joint attention in parent-child dyads involving children with selective mutism: a comparison between anxious and typically developing children.

PubMed

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

2011-02-01

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 measures coded from direct observations with their parent during an unstructured free play task and two structured tasks. As predicted, the SM dyads established significantly fewer episodes of joint attention through parental initiation acts than the MA and CC dyads during the structured tasks. Findings suggest that children with SM may withdraw from their parents during stressful situations, thus missing out on opportunities for learning other coping skills. We discuss the implications of the present findings for understanding the maintenance and treatment of SM.

Selective mutism: a team approach to assessment and treatment in the school setting.

PubMed

Ponzurick, Joan M

2012-02-01

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. Early diagnosis and treatment provide the key to addressing this rare disorder. The school nurse plays a critical role as a member of the Instructional Support Team (IST). The school nurse, as team liaison, provides communication between parents, school staff, and medical personnel. School nurses make a difference by advocating for the child with SM and possessing the necessary knowledge to effectively intervene. This article discusses a team approach to the assessment and treatment of SM and the role of the school nurse in the school setting.

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

PubMed

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.

Comparing interventions for selective mutism: a pilot study.

PubMed

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.

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

PubMed

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.

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

PubMed

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.

Acupuncture and Deaf-Mutism--An Essay in Cross-Cultural Defectology

ERIC Educational Resources Information Center

Sutton, Andrew

1977-01-01

Describes reportedly successful treatment of deaf-mutes in China by acupuncture, and suggests that the disorders might have been more psychological than physical. The author comments that interpretation of poorly documented reports from unfamiliar cultures is extremely difficult. (AV)

Cannabis Induced Periodic Catatonia: A Case Report

ERIC Educational Resources Information Center

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…

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

PubMed Central

Eugene, Andy R.; Masiak, Jolanta

2016-01-01

Background 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. Methods 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). Results 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. Conclusion Our findings suggest that electrophysiological neuroimaging may provide a creative approach for personalizing medicine by providing insight to the pharmacodynamics of antidepressants. PMID:27468379

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

PubMed

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.

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

PubMed

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.

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

PubMed

Østergaard, Kasper Rud

2018-05-01

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.

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

PubMed

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.

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

PubMed

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.

Evaluation of children with selective mutism and social phobia: a comparison of psychological and psychophysiological arousal.

PubMed

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

2012-07-01

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), or no diagnosis (n = 14) participated in the current study. Measurements included clinician, child, and parent ratings as well as behavioral observations and psychophysiological measures. Independent evaluators and clinicians rated children with SM as more severely impaired, more anxious, and less socially effective, but the groups did not differ in self- or parent-reported anxiety. Psychophysiological measures indicated that children in the SM group experienced less arousal than other children during social interaction tasks. The authors postulate that lack of speech may serve as an avoidance mechanism and thus account for this lack of arousal.

Goiter and deaf mutism.

PubMed

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.

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

PubMed

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.

Major Depression in a Small Group of Adults with Down Syndrome.

ERIC Educational Resources Information Center

Myers, Beverly A.; Pueschel, Siegfried M.

1995-01-01

The clinical histories and treatment of 9 individuals with Down syndrome and major depression are presented, as are clinical characteristics of an additional 13 individuals. Vegetative symptoms of disinterest, withdrawal, mutism, psychomotor retardation, decreased appetite, and insomnia were prominent. Preoccupations with suicide, death,…

Using Behavioral Techniques to Treat Elective Mutism: A Case Study.

ERIC Educational Resources Information Center

Richburg, Mary L.; Cobia, Debra C.

1994-01-01

Describes treatment using combination of behavioral techniques, contingency management with positive reinforcement, and stimulus fading for kindergarten girl who was referred to school counselor because of her unwillingness to verbally communicate with her teacher and peers. Discusses how counseling techniques produced desired outcomes, expanding…

Intervention with the Selectively Mute Child.

ERIC Educational Resources Information Center

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.…

Narrative Counseling for Professional School Counselors

ERIC Educational Resources Information Center

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…

Catatonia in Autism: A Distinct Subtype?

ERIC Educational Resources Information Center

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

2005-01-01

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

Silent at school--elective mutism and abuse.

PubMed Central

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

Interviews with Selectively Mute Children

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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…

Some Generalization and Follow-Up Measures on Autistic Children in Behavior Therapy.

ERIC Educational Resources Information Center

Lovaas, O. Ivar; And Others

Reported was a behavior therapy program emphasizing language training for 20 autistic children who variously exhibited apparent sensory deficit, severe affect isolation, self stimulatory behavior, mutism, echolalic speech, absence of receptive speech and social and self help behaviors, and self destructive tendencies. The treatment emphasized…

Establishing Vocal Verbalizations in Mute Mongoloid Children.

ERIC Educational Resources Information Center

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,…

Elective Mutism--A Success Story.

ERIC Educational Resources Information Center

Caputo, Norma; Crawford, Margie

This paper presents a case study of successful intervention with a boy (in fourth grade at the time of the intervention) who would not speak in the school environment. Intervention involved medication with Fluoxetine to relieve anxiety, parental involvement, and a six-step behavioral therapy plan of gradually increasing speech demands in the…

Huntington Disease: A Case Study of Early Onset Presenting as Depression

ERIC Educational Resources Information Center

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

2004-01-01

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…

Without a Voice

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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)

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

PubMed

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.

A long-term outcome study of selective mutism in childhood.

PubMed

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

2006-07-01

Controlled study of the long-term outcome of selective mutism (SM) in childhood. 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 childhood. The three groups were compared with regard to psychiatric disorder in young adulthood by use of the Composite International Diagnostic Interview (CIDI). In addition, the effect of various predictors on outcome of SM was studied. The symptoms of SM improved considerably in the entire SM sample. However, both SM and ANX had significantly higher rates for phobic disorder and any psychiatric disorder than controls at outcome. Taciturnity in the family and, by trend, immigrant status and a severity indicator of SM had an impact on psychopathology and symptomatic outcome in young adulthood. This first controlled long-term outcome study of SM provides evidence of symptomatic improvement of SM in young adulthood. However, a high rate of phobic disorder at outcome points to the fact that SM may be regarded as an anxiety disorder variant.

Behavioral and socio-emotional functioning in children with selective mutism: a comparison with anxious and typically developing children across multiple informants.

PubMed

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

2010-11-01

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 lower than controls on a range of social skills, but the SM and MA groups did not significantly differ on many of the social skills and anxiety measures. However, children with SM were rated higher than children with MA and controls on social anxiety. Findings suggest that SM may be conceptualized as an anxiety disorder, with primary deficits in social functioning and social anxiety. This interpretation supports a more specific classification of SM as an anxiety disorder for future diagnostic manuals than is currently described in the literature. The present findings also have implications for clinical practice, whereby social skills training merits inclusion in intervention for children with anxiety disorders as well as children with SM.

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

PubMed Central

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

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

PubMed

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%.

Goiter and deaf mutism.

PubMed Central

Thieme, E T

1975-01-01

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. Images Fig. 1. Fig. 2. Fig. 3. Fig. 4. Fig. 5. PMID:1211996

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

PubMed

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.

Afraid To Be Heard: The Selectively Mute Child.

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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…

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

ERIC Educational Resources Information Center

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…

Selective mutism: more than social anxiety?

PubMed

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.

Selective mutism and social anxiety disorder: all in the family?

PubMed

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

2007-11-01

To examine the history of lifetime psychiatric disorders in the parents of children with selective mutism (SM) compared to parents of children in a control group. Seventy parent dyads (n = 140) of children with lifetime SM and 31 parent dyads (n = 62) of children without SM were interviewed with the Structured Clinical Interview for DSM-IV (IV and II) anxiety disorders, mood disorders, avoidant personality disorder, and schizoid personality disorder modules via telephone. Interviewers were blind to proband status. The NEO Personality Inventory was also administered. Lifetime generalized social phobia was present in 37.0% of SM parents compared to 14.1% of control parents (chi2 = 10.98; p < .001; odds ratio 3.6, 95% confidence interval 1.6-7.9). Avoidant personality disorder was present in 17.5% of the SM parents compared to 4.7% of control parents (chi2 = 6.18; p < .05; odds ratio 4.3, 95% confidence interval 1.3-14.9). The proportion of parents with other psychiatric disorders was not different between groups. SM parents had higher neuroticism and lower openness scores on the NEO Personality Inventory than control parents. These results support earlier uncontrolled findings of a familial relationship between generalized social phobia and SM.

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

PubMed

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.

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

PubMed

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.

Behavioral and emotional adjustment, family functioning, academic performance, and social relationships in children with selective mutism.

PubMed

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

2004-11-01

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 52 community controls, 52 children with SM were more anxious, obsessive, and prone to somatic complaints. In contrast, children with SM were less oppositional and evidenced fewer attentional difficulties at school. We found no group differences in family structure, economic resources, family functioning, maternal mood difficulties, recreational activities, or social networks. While parents reported no differences in parenting strategies, children with SM were described as less cooperative in disciplinary situations. The academic (e.g., reading and math) and classroom cooperative skills of children with SM did not differ from controls. Parents and teachers reported that children with SM had significant deficits in social skills. Though teachers and parents rated children with SM as less socially assertive, neither teachers nor parents reported that children with SM were victimized more frequently by peers.

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

PubMed

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.

Advanced Cancer Detection Center

DTIC Science & Technology

2007-10-01

therapy in children: A Phase II randomized double blinded cross-over study (HLMCC 0708) • Risperidone for the Treatment of Cerebellar Mutism Syndrome...each question, the participant selects the answer by using the left and right arrow keys and then pressing the Choose button. The next question is...transformed to a quantitative scale measure for transmission. Numeric responses can be entered directly or can be selected from a list of

HTLV-III: Intra-BBB IgG Synthesis and Hybridization in CSF Cells

DTIC Science & Technology

1989-01-31

neuropsychologic testing in otherwise asymptomatic individuals [ 9], to a bedridden state marked by global dementia, severe hypokinesis, mutism , incontinence...could result from infection by more than one strain, mutation in vivo, viral adaptation, or host cell selection from a heterogeneous virus population...lower than ours -personal communication, Marshall, Nov. 1988), and their selection by military recruitment personnel for overall good physical health. The

Speech Rate as a Sticky Switch: A Multiple Lesion Case Analysis of Mutism and Hyperlalia

ERIC Educational Resources Information Center

Braun, Claude M. J.; Dumont, Mathieu; Duval, Julie; Hamel-Hebert, Isabelle

2004-01-01

Though it has long been known on the basis of clinical associations and serendipitous observation that speech rate is related to mood and psychomotor baseline, it is less known that speech rate is also related to libido and to immune function. We make the case for a bipolar phenomenon of ''psychic tonus,'' encompassing all these dimensions. The…

Behavioral Consequences of Kainic Acid Lesions and Fetal Transplants of the Striatum

DTIC Science & Technology

1984-06-12

Selected sections were also stained with cresyl violet in order to facilitate the visualization of neuronal cytology and morphology. All sections...tendency to mutism and depression with frequent suicidal ideation (Bruyn, 1973). The Westphal variant of HD, also called the rigid-hypokinetic...1978). In situ injections of kainic acid: A new method for selectively lesioning neuronal cell bodies while sparing axons of passage. Journal of

European Scientific Notes, Volume 38, Number 9.

DTIC Science & Technology

1984-09-01

dropped automa- tically from the mailing list. RSN Invites Letters to the Editor ESN publishes selected letters related to developments and policy in... selective sunmmary can be extract- examine trait anxiety or state-trait ed from the Idzikowski-Baddeley litera- interactions. ture review; it appears in... mutism , and stupor are not seen in fliers as they are in ground soldiers. Reid 1945 WW II - Navigation Errors increased over enemy bomber errors coast

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

PubMed

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.

P50 suppression in children with selective mutism: a preliminary report.

PubMed

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 children with SM. The classic paired-click paradigm was utilized to assess suppression of the P50 event-related potential to the second, of two sequentially-presented clicks, in ten children with SM and 10 control children. A significant suppression of P50 to the second click was evident in the SM group, whereas no suppression effect was observed in controls. Suppression was evident in 90% of the SM group and in 40% of controls, whereas augmentation was found in 10% and 60%, respectively, yielding a significant association between group and suppression of P50. P50 to the first click was comparable in children with SM and controls. The adult-like, mature P50 suppression effect exhibited by children with SM may reflect a cortical mechanism of compensatory inhibition of irrelevant repetitive information that was not properly suppressed at lower levels of their auditory system. The current data extends our previous findings suggesting that differential auditory processing may be involved in speech selectivity in SM.

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

PubMed

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.

Creutzfeldt-Jakob Disease Presenting With Dizziness and Gaze-Evoked Nystagmus: A Case Report.

PubMed

Choi, Yun-Ju; Kang, Kyung-Wook; Lee, Sae-Young; Kang, Seung-Ho; Lee, Seung-Han; Kim, Byeong C

2016-02-01

Sporadic Creutzfeldt-Jakob disease (CJD) is clinically characterized by rapidly progressive dementia combined with other cardinal symptoms, such as myoclonus, visual or cerebellar disturbances, extrapyramidal or pyramidal disturbance, and akinetic mutism. However, as an initial manifestation, focal neurologic deficits other than the aforementioned or nonspecific generalized symptoms may lead to a misdiagnosis or a delayed diagnosis. The authors report a case of 66-year-old male patient with sporadic CJD who had dizziness, gaze-evoked nystagmus (GEN), and other central eye signs (impaired smooth pursuit, saccadic dysmetria) as an initial manifestation without dementia. The central eye signs led us to perform brain magnetic resonance images, which showed abnormal cortical high-signal intensity in both the cerebral and cerebellar hemispheres including the vestibulocerebellum. We reached a presumptive diagnosis of CJD, but the findings did not meet diagnostic criteria for probable CJD at that time. Three weeks after the initial work-ups, the patient presented with typical neurological findings of CJD: rapidly progressive dementia, akinetic mutism, and myoclonus of the left arm. Cerebrospinal fluid was positive for 14-3-3 protein, and electroencephalography showed periodic sharp wave complexes. In this patient, GEN and other central eye signs provided diagnostic clues for CJD. These unusual neurological manifestations may help physicians have a thorough knowledge of early deficits of CJD.

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

ERIC Educational Resources Information Center

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…

Proceedings: User’s Workshop on Combat Stress Held at Fort Sam Houston, Texas on 2-4 September 1981.

DTIC Science & Technology

1983-12-01

Primary Treatment Principle * Clinical Picture: Tremors, Paralysis, Mutism , Ganser Syndrome 5. WW II * No Effective Treatment-Evacuation Policy Existed...there is a lack of specific detailed tasks and the amount of time expended in these tasks for selected specialty skill identifiers. Information is...The study objective is the identification of the various tasks to be per- formed by selected specialty skill identifiers dealing with psychiatric

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

PubMed

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.

Childhood disintegrative disorder with seasonal total mutism: A rare clinical presentation

PubMed Central

Shirazi, Elham; Hosseinpoor, Sara; Mirhosseini, Seyyed Mohammad Mahdy; Bidaki, Reza

2016-01-01

Childhood disintegrative disorder (CDD) is a rare autistic-like clinical condition with unknown etiology, in that previously acquired age-appropriate language, social and adaptive abilities deteriorate significantly in 2-10-year-old healthy children, although physical and neurological evaluations display no observable abnormality. Our case is a 22-year-old female born of a consanguineous marriage, with the appearance of CDD symptoms in her fifth year of age following normal mental and physical development during her initial four years of life. Without any precipitating factor, she gradually lost her language abilities, social relational skills, affectionate behavior, adaptive capacities, peer play and meaningful interest in her surrounding, friends and family members over a period of 4 years, reaching a plateau in her ninth year of age. The unique special clinical symptom in this case is a seasonal total mutism, which after the beginning of her CDD symptoms is revealing every year covering the spring. As no additional physical or psychological change accompanies her total seasonal speech loss, it cannot be attributed to any mental condition known as having a seasonal pattern. Because in the literature CDD is presented mostly as case reports with lacking of advanced research data, describing any new case is recommended to improve the knowledge about this rare condition, especially if it displays some new unusual signs, not reported till now. PMID:27069898

Selective Mutism: The Fraternal Twin of Childhood Social Phobia.

PubMed

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.

[Mutism and acute behavioral disorders revealing MELAS syndrome].

PubMed

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.

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

PubMed

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.

Proceedings: Second User’s Workshop on Combat Stress Held at Fort Sam Houston, Texas on 29-30 April 1982.

DTIC Science & Technology

1983-12-01

participant spaces are limited, selection for attendance will be based upon the following priorities: a. Unit is willing to have both line officers with...Proximity Re-Emerged as Primary Treatment Principle. * Clinical Picture: Tremors, Paralvsis, Mutism , Ganser Syndrome. 5. WW II: * lo Effective Treatment...aspects of the job, not on the danger. A soldier who is concentrating on firing his weacon and on selecting his next firing position is not concentrating

Subacute sclerosing panencephalitis. Changes on CT scan during acute relapse.

PubMed

Modi, G; Campbell, H; Bill, P

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.

Effects of anxiety, language skills, and cultural adaptation on the development of selective mutism.

PubMed

Starke, Anja

Although bilingual children are thought to be at higher risk for selective mutism (SM), little is known about the development of SM in this population. This study investigates the effects of children's anxiety and language skills and parents' cultural adaptation on the development of SM. 15 bilingual (11 mute, 4 speaking at the beginning of the study) and 15 monolingual children (7 mute, 8 speaking at the beginning of the study) between the ages of 3 years and 5 years 8 months were assessed longitudinally over a 9-month period. Children's anxiety and parents' cultural adaptation were examined via parent questionnaires. Receptive language skills were assessed with a standardized test. Every 3 months, parents and preschool teachers reported on the children's speaking behavior via questionnaires. Anxiety best predicted the development of mute behavior. There was no effect of bilingual status on its own. The effect of language skills did not reach significance but was considerably higher in preschool settings in comparison with family and public situations. Results also indicated an association between parents' orientation to the mainstream culture and children's speaking behavior in preschool. Level of anxiety might function as an early indicator of SM, especially in bilingual children, when information on language proficiency is scarce. There is still a need for intensive research in order to further the understanding of the development of SM in bilingual children. Copyright © 2018 Elsevier Inc. All rights reserved.

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

PubMed

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.

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

PubMed

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

2018-06-01

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.

[The otorhinolaryngological achievements of surgeon Hipolit Korzeniowski (1827-1897)].

PubMed

Kierzek, Andrzej

2006-01-01

The professional and scientific activities of Hipolit Korzeniowski (1827-1879), an eminent Varsovian surgeon, his thorough medical education in various European centries is shortly outlined. He was a head of the surgical ward especially at the Infant Jesus Hospital in Warsaw and at the medical schools in Warsaw. Korzeniowski's succesful surgical treatment of rhinolaryngological diseases, such as scarlet fever of ear, treatment of deaf-mutism, sinusitis paranasales, operations of jaws, plastic reconstruction of the palate, foreign bodies of larynx, tracheotomy in the tuberculosis of larynx, are described in some more detail.

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

PubMed

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

2014-06-01

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

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

PubMed

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.

Comparison of Behavioral Profiles for Anxiety-Related Comorbidities including ADHD and Selective Mutism in Children

PubMed Central

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

2013-01-01

Background 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. Methods 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). Results 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 problem social behaviors than either 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. Conclusions 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. PMID:23526795

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

PubMed

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.

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

PubMed

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.

Austistic Children: Bodily Factors in the Use of Language.

PubMed

Rhode, Maria

2015-01-01

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

A Common Genetic Variant in the Neurexin Superfamily Member CNTNAP2 is Associated with Increased Risk for Selective Mutism and Social Anxiety-Related Traits

PubMed Central

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

2010-01-01

Background Selective mutism (SM), considered an early-onset variant of social anxiety disorder (SAD), shares features of impaired social interaction and communication with autism spectrum disorders (ASDs) that suggest a possible shared pathophysiology. We examined the association of a susceptibility gene, contactin-associated protein-like 2 (CNTNAP2), for ASDs and specific language impairment (SLI) with SM and social anxiety-related traits. Methods 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 (SIAS; N = 1028) and childhood behavioral inhibition (RSRI; N = 920). Five SNPs in CNTNAP2 (including rs7794745 and rs2710102, previously associated with ASDs) were genotyped. Results FBAT analyses revealed nominal significance (p = 0.018) for association of SM with rs2710102 which, with rs6944808, was part of a common haplotype associated with SM (permutation p = 0.022). 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 >1SD above the mean on the SIAS (OR = 1.33, p = 0.015) and RSRI (OR = 1.40, p = 0.010). Discussion Although association was found with rs2710102, the risk allele (“a”) for the traits studied here is the non-risk allele for ASD and SLI (“g”). These findings suggest a partially shared etiology between ASDs and SM, but raise additional questions about specific aspects of these syndromes (i.e., language impairment and/or social anxiety) potentially influenced by CNTNAP2 and mechanism(s) by which these influences may be conveyed. PMID:21193173

Selective mutism: are primary care physicians missing the silence?

PubMed

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.

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

PubMed

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.

Abnormalities in auditory efferent activities in children with selective mutism.

PubMed

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.

Phenomenology and treatment of Catatonia: A descriptive study from north India

PubMed Central

Dutt, Alakananda; Grover, Sandeep; Chakrabarti, Subho; Avasthi, Ajit; Kumar, Suresh

2011-01-01

Background: Studies on clinical features of catatonia in the Indian population are few in number. Aim: To study the phenomenology, clinical profile and treatment response of subjects admitted to the psychiatry inpatient with catatonia. Materials and Methods: Detailed treatment records of all the inpatients were scanned for the period January 2004 to December 2008. Patients with catatonia (diagnosed as two symptoms as per the Bush Francis Catatonia Rating scale) were included. Results: During the study period, 1056 subjects were admitted in the inpatient unit, of which 51 (4.8% of the total admissions) had catatonic features and had been rated on the Bush Francis Catatonia Rating scale. The mean age of the sample was 30.02 years (SD=14.6; range 13-69), with an almost equal gender ratio. Most of the patients presenting with catatonia were diagnosed as having psychotic disorders (40; 74.8%), of which the most common diagnosis was schizophrenia (27; 52.9%) of the catatonic subtype (20; 39.2%). Three subjects with primary diagnosis of a psychotic disorder had comorbid depression. Other diagnoses included mood disorders (7; 13.72%) and organic brain syndromes (04; 7.9%). According to the Bush Francis Rating scale, the common signs and symptoms exhibited by the subjects were mutism (94.1%), followed by immobility/stupor (78.5%), staring (78.4%), negativism (74.5%), rigidity (63%) and posturing/catalepsy (61.8%). All the patients were initially treated with lorazepam. Electroconvulsive therapy was required in most cases (42; 82.35%). Conclusion: The common symptoms of catatonia are mutism, immobility/stupor, staring, posturing, negativism and rigidity. The most common underlying psychiatric diagnosis was schizophrenia. PMID:21431006

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

PubMed

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.

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.

PubMed

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".

PubMed

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.

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

PubMed

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 no-pCMS group, significantly more reported incidents of severe bleeding in the tumor bed during surgery in the pCMS group, preoperative hydrocephalus, and a mean body temperature rise of 0.5°C in the first 4 days after surgery in the pCMS group. Multiple regression analysis revealed that tumor size, tumor infiltration into or compression of the brainstem, and higher mean body temperature in the first 4 postoperative days were independent and highly significant predictors for pCMS. CONCLUSIONS The authors confirmed earlier findings that tumor-associated preoperative conditions, such as a maximum tumor diameter ≥ 5 cm and infiltration into or compression of the brainstem, are associated with a higher risk for the development of pCMS. Most importantly, the authors found that a 0.5°C higher mean body temperature in the first 4 postoperative days increased the odds ratio for the development of pCMS almost 5-fold. These data suggest that an important focus for the prevention of pCMS in children who have undergone medulloblastoma surgery might be rigorous maintenance of normothermia as standard care after surgery.

Catatonic syndrome associated with lead intoxication: a case report.

PubMed

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.

MRI and clinical manifestations of delayed encephalopathy after carbon monoxide poisoning.

PubMed

Wang, Xiahong; Li, Zhenyu; Berglass, Jacqueline; He, Wenlong; Zhao, Jianmin; Zhang, Min; Gao, Chongyang; Zhang, Caixia; Zhang, Huimin; Yi, Xuewei

2016-11-01

To explore the relationship between the clinical manifestations and functional magnetic resonance images of delayed encephalopathy after carbon monoxide intoxication. Six patients received the MRI were diagnosed with delayed encephalopathy after carbon monoxide (CO) poisoning. Clinical manifestations were observed in each patient. MRI revealed multiple lesions. The majority of the lesions were located in the globus pallidus, sub cortical white matter, and basal ganglia. The cognitive injury, akinetic mutism, fecal and uroclepsia, forced crying, forced laughing and extra pyramidal syndromes such as chorea and parkinsonism were manifested in clinic. Cognitive impairment improved greatly while involuntary movements only improved slightly after several months. Meanwhile brain MRI suggested remarkable improvement. Neuroimaging directly correlated with the clinical manifestations.

[Catatonia in the elderly].

PubMed

Chiba, Yuhei; Odawara, Toshinari

2013-10-01

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

Nothing new under the sun: post-traumatic stress disorders in the ancient world.

PubMed

Abdul-Hamid, Walid Khalid; Hughes, Jamie Hacker

2014-01-01

Herodotus' account of the Athenian spear carrier Epizelus' psychogenic mutism following the Marathon Wars is usually cited as the first documented account of post-traumatic stress disorders in historical literature. This paper describes much earlier accounts of post combat disorders that were recorded as occurring in Mesopotamia (present day Iraq) during the Assyrian dynasty (1300-609 BC). The descriptions in this paper include many symptoms of what we would now identify in current diagnostic classification systems as post-traumatic stress disorders; including flashbacks, sleep disturbance and low mood. The Mesopotamians explain the disorder in terms of spirit affliction; the spirit of those enemies whom the patient had killed during battle causing the symptoms.

[Anxiety disorders in DSM-5].

PubMed

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.

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

PubMed Central

Potgieser, A. R. E.; de Jong, B. M.; 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 feature is that these symptoms completely resolve within weeks to months, leaving only a disturbance in alternating bimanual movements. In this review we give an overview of the old and new insights from the SMA syndrome and extrapolate these findings to seemingly unrelated diseases and symptoms such as Parkinson’s disease (PD) and tics. Furthermore, we integrate findings from lesion, stimulation and functional imaging studies to provide insight in the motor function of the SMA. PMID:25506324

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

PubMed

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.

N-methyl-d-aspartate (NMDA) receptor antibodies encephalitis mimicking an autistic regression.

PubMed

Hacohen, Yael; Wright, Sukhvir; Gadian, Jonathan; Vincent, Angela; Lim, Ming; Wassmer, Evangeline; Lin, Jean-Pierre

2016-10-01

Expressive dysphasia and mutism are common clinical features in children and adults with N-methyl-d-aspartate receptor antibodies (NMDAR-Ab) encephalitis, and are likely to result from NMDAR hypofunction. A prodromal loss of social and communication skills can typify that of an autistic regression, particularly when presenting under the age of 3 years. Here we describe two toddlers who presented with developmental regression, particularly of their social communication skills, mimicking an autistic regression, who were found to have NMDAR-Ab in the serum and cerebrospinal fluid. Although both patients had some other neurological features, they were subtle, which resulted in delayed diagnosis of NMDAR-Ab encephalitis. Importantly, immunotherapy was beneficial in both patients, with significant improvement of their language skills and behaviour. © 2016 Mac Keith Press.

Multi-Modal Ultra-Widefield Imaging Features in Waardenburg Syndrome

PubMed Central

Choudhry, Netan; Rao, Rajesh C.

2015-01-01

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

Renal Failure in Dementia with Lewy Bodies Presenting as Catatonia

PubMed Central

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

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

PubMed

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 reached as to what constitutes clinical progress, intervals for monitoring progress, criteria for referral onwards for multidisciplinary specialist assessment and the role of selective serotonin reuptake inhibitor (SSRI) medication. A consensus methodology has been successfully used to compensate for the lack of evidence base and harness the expertise of a relatively small number of experienced professionals in order to provide a basis for the future development of services.

Consciousness: Its Neurobiology and the Major Classes of Impairment

PubMed Central

Goldfine, Andrew M.; Schiff, Nicholas D.

2011-01-01

Summary Normal human consciousness requires brainstem, basal forebrain, and diencephalic areas to support generalized arousal, as well as functioning thalamocortical networks to become aware of, and respond to environmental and internal stimuli. Injury to or disconnection of these interconnected systems, typically from cardiac arrest and traumatic brain injury, can result in disorders of consciousness, including coma, vegetative state, minimally conscious state, and akinetic mutism. Similar brain injuries can also result in loss of motor output out of proportion to consciousness, resulting in misdiagnoses of disorders of consciousness. We review pathology and imaging studies and derive mechanistic models for each of these conditions, to aid in the assessment and prognosis of individual patients. We further suggest how such models may guide the development of target-based treatment algorithms to enhance recovery of consciousness in many of these patient. PMID:22032656

Japanese encephalitis in a French traveler to Nepal.

PubMed

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.

Can autism spectrum disorders and social anxiety disorders be differentiated by the social responsiveness scale in children and adolescents?

PubMed

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

2014-05-01

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 typically developed (N = 42). Sensitivity and specificity were examined. The three disorders showed overlapping SRS scores. Especially in boys with SM (ROC-AUC = .81), presence of ASD was overestimated by the SRS. A combination of three disorder specific questionnaires resulted in marginally improved diagnostic accuracy. For the clinically very relevant differential diagnosis of SP/SM, SRS results must be interpreted with caution.

Results of quinacrine administration to patients with Creutzfeldt-Jakob disease.

PubMed

Nakajima, Masashi; Yamada, Tatsuo; Kusuhara, Tomohiko; Furukawa, Hisako; Takahashi, Mitsuo; Yamauchi, Atsushi; Kataoka, Yasufumi

2004-01-01

Several chemicals inhibit the accumulation of abnormal prion proteins in vitro. We administered one, the antimalarial agent quinacrine, to three patients with sporadic Creutzfeldt-Jakob disease (CJD) and to one with iatrogenic CJD. Quinacrine at 300 mg/day was given enterally for 3 months. Within 2 weeks of administration, the arousal level of the patient with akinetic mutism improved. The other 3 patients, insensible before treatment, had integrative responses such as eye contact or voluntary movement in response to verbal and/or visual stimuli restored. Clinical improvement was transient, lasting 1-2 months during treatment. Quinacrine was well tolerated, except for liver dysfunction and yellowish pigmentation. Although its antiprion activity in the human brain has yet to be proved, these modest effects of quinacrine suggest the possibility of using chemical intervention against prion diseases. Copyright 2004 S. Karger AG, Basel

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

PubMed

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.

Aspects of the analysis of an adult son of deaf-mute parents.

PubMed

Wagenheim, H S

1985-01-01

This case demonstrates unique features connected with the parental deafness: the patient's anger toward women, his splitting of objects into all-good and all-bad, ease of regression, yearning for the symbiotic mother particularly as it was related to space as a transitional phenomenon into which he brooked no intrusion, his unique interests and creativity, his method of teaching, the shaping of the Oedipus complex by preoedipal trauma, and his feeling of entitlement with need to be treated as an exception. I have attempted to demonstrate the relation of the preverbal and preoedipal traumata, stemming from the deaf-mutism of the parents of a hearing child, to the patient's pathology, the shaping of his oedipal constellation, and character traits. I have described the compensatory ego functions that resulted in the development of creative, professional ability. Also, I have tried to demonstrate the complicated multiple overdetermination of his symptoms and aspects of his character formation.

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

PubMed

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.

Abnormal laughter-like vocalisations replacing speech in primary progressive aphasia

PubMed Central

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

Neurologic Complications of Transplantation.

PubMed

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.

Abnormal laughter-like vocalisations replacing speech in primary progressive aphasia.

PubMed

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

2009-09-15

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.

Clozapine in schizophrenia patients with recurrent catatonia: report of two cases.

PubMed

Hung, Yi-Yung; Yang, Ping-Suen; Huang, Tiao-Lai

2006-04-01

Prolonged catatonia can be a source of extremely serious morbidity and mortality. Lorazepam is effective in rapidly relieving most cases of catatonia. Reports have also shown that second-generation antipsychotic drugs are also efficacious in relieving catatonia. This report describes two schizophrenia patients who demonstrated recurrent catatonic features mutism and stupor. Both patients were treated with lorazepam, diazepam or electroconvulsive therapy (ECT). Patient 1 responded well and rapidly to lorazepam each time catatonia happened; but catatonia recurred once a year under treatment with many antipsychotic drugs. Patient 2 had catatonia features associated with discontinuing or decreasing clozapine. With each recurrent episode, the duration of catatonia increased, requiring an increased dosage of benzodiazepine. The patient's response to lorazepam and ECT gradually decreased, until the patient had almost no response to lorazepam, diazepam or ECT. Both patients had no recurrence during a period of 2-year follow up with continuous clozapine therapy.

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

PubMed

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.

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

PubMed

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.

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

PubMed Central

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

Catatonia is not schizophrenia and it is treatable.

PubMed

Appiani, Francisco J; Castro, Gonzalo S

2017-06-10

Catatonia is a cluster of motor features that appears in many recognized psychiatric illnesses, that according to the DSM-5 it is not linked as a subtype to schizophrenia anymore. The classic signs are mutism, a rigid posture, fixed staring, stereotypic movements, and stupor, which are all part of a broad psychopathology that may be found in affective, thought, neurological, toxic, metabolic and immunological disorders. Despite the many etiologies, catatonia may be a life-threatening condition with a specific treatment. Benzodiazepines are the first line therapeutic option for catatonia, being lorazepam the first-choice drug. Eighty percent of the patients are relieved by the use of barbiturates or benzodiazepines, while in those who fail, an improvement is achieved by electroconvulsive therapy (ECT). With more than 60years of use in catatonic patients, ECT has proven to be an effective and safe tool for the treatment of this frequent and sometimes forgotten syndrome. Copyright © 2017 Elsevier B.V. All rights reserved.

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

PubMed

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.

Contralateral functional reorganization of the speech supplementary motor area following neurosurgical tumor resection.

PubMed

Chivukula, Srinivas; Pikul, Brian K; Black, Keith L; Pouratian, Nader; Bookheimer, Susan Y

2018-05-18

We evaluated plasticity in speech supplemental motor area (SMA) tissue in two patients using functional magnetic resonance imaging (fMRI), following resection of tumors in or associated with the dominant hemisphere speech SMA. Patient A underwent resection of a anaplastic astrocytoma NOS associated with the left speech SMA, experienced SMA syndrome related mutism postoperatively, but experienced full recovery 14 months later. FMRI performed 32 months after surgery demonstrated a migration of speech SMA to homologous contralateral hemispheric regional tissue. Patient B underwent resection of a oligodendroglioma NOS in the left speech SMA, and postoperatively experienced speech hesitancy, latency and poor fluency, which gradually resolved over 18 months. FMRI performed at 64 months after surgery showed a reorganization of speech SMA to the contralateral hemisphere. These data support the hypothesis of dynamic, time based plasticity in speech SMA tissue, and may represent a noninvasive neural marker for SMA syndrome recovery. Copyright © 2018 Elsevier Inc. All rights reserved.

Vegetative state, minimally conscious state, akinetic mutism and Parkinsonism as a continuum of recovery from disorders of consciousness: an exploratory and preliminary study

PubMed Central

Formisano, Rita; D’Ippolito, Mariagrazia; Risetti, Monica; Riccio, Angela; Caravasso, Chiara Falletta; Catani, Sheila; Rizza, Federica; Forcina, Antonio; Buzzi, Maria Gabriella

Summary The aim of this study was to review the usefulness of clinical and instrumental evaluation in individuals with disorders of consciousness (DOC). Thirteen subjects with severe acquired brain injury (ABI) and a diagnosis of DOC were evaluated using the Coma Recovery Scale in its revised version (CRS-R) and a new global disability index, the Post-Coma Scale (PCS). These instruments were administered both by a neutral examiner (professional) and by a professional in the presence of a caregiver. All patients were also scored using the International Classification of Functioning, Disability and Health (ICF). A statistically significant correlation between CRS-R and PCS was demonstrated. However, there also emerged significant differences in responsiveness between professional versus caregiver+professional assessment using the two scales. The emotional stimulation provided by significant others (caregivers) during administration of DOC evaluation scales may improve the assessment of responsiveness. PMID:21693084

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

PubMed

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.

Special Medical Conditions Associated with Catatonia in the Internal Medicine Setting: Hyponatremia-Inducing Psychosis and Subsequent Catatonia

PubMed Central

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

2014-01-01

Diagnosis and treatment of catatonia in the psychiatry consultation service is not infrequent. Usually, the patient either presents to the Emergency Department or develops catatonia on the medical floor. This condition manifests with significant behavioral changes (from mildly decreased speech output to complete mutism) that interfere with the ability to communicate. After structural brain disorders are excluded, one of the diagnoses that always should be considered is catatonia. However, the causes of catatonia are numerous, ranging from psychiatric causes to a plethora of medical illnesses. Therefore, it is not surprising that there are many proposed underlying mechanisms of catatonia and that controversy persists about the etiology of specific cases. There are only 6 reports of hyponatremia-induced catatonia and psychosis in the literature. Here, we present the case of a 30-year-old woman with catatonia and psychosis induced by hyponatremia, and we use this report to exemplify the multitude of biologic causes of catatonia and to propose a new way to look at the neuroanatomical basis of processing, particularly the vertical processing systems we believe are involved in catatonia. PMID:25102520

Using human extra-cortical local field potentials to control a switch

NASA Astrophysics Data System (ADS)

Kennedy, Philip; Andreasen, Dinal; Ehirim, Princewill; King, Brandon; Kirby, Todd; Mao, Hui; Moore, Melody

2004-06-01

Individuals with profound paralysis and mutism require a communication channel. Traditional assistive technology devices eventually fail, especially in the case of amyotrophic lateral sclerosis (ALS) subjects who gradually become totally locked-in. A direct brain-to-computer interface that provides switch functions can provide a direct communication channel to the external world. Electroencephalographic (EEG) signals recorded from scalp electrodes are significantly degraded due to skull and scalp attenuation and ambient noise. The present system using conductive skull screws allows more reliable access to cortical local field potentials (LFPs) without entering the brain itself. We describe an almost locked-in human subject with ALS who activated a switch using online time domain detection techniques. Frequency domain analysis of his LFP activity demonstrates this to be an alternative method of detecting switch activation intentions. With this brain communicator system it is reasonable to expect that locked-in, but cognitively intact, humans will always be able to communicate. Financial disclosure. Authors PK and DA may derive some financial gain from the sale of this device. A patent has been applied under US and international law: 10/675,703.

Catatonic syndrome: importance of detection and treatment with lorazepam.

PubMed

Seethalakshmi, R; Dhavale, S; Suggu, Kalpana; Dewan, Mantosh

2008-01-01

A resurgence of interest has led to renewed attempts to clarify the concept and treatment of catatonia. A large prospective study was conducted to estimate the incidence of catatonic syndrome in 138 consecutive psychiatric patients admitted to a general hospital in India, to demarcate the common symptom presentations and its response to intravenous benzodiazepines. Patients were screened using the Bush Francis Catatonia Screening Instrument. Patients with two or more signs on the Instrument were subsequently administered intravenous lorazepam and their response was rated on the Bush Francis Catatonia Rating Scale. Catatonic syndrome was found in 11% of patients with a wide variety of diagnoses, especially schizophrenia. Mutism (87.5% incidence) was the most common symptom. A significant proportion (93%) of these patients showed a marked immediate response to lorazepam, with 75% showing sustained improvement. Catatonic syndrome is common, often undiagnosed, and quickly responsive to treatment, irrespective of the diagnosis. It needs to be identified and actively treated with benzodiazepines to minimize distress, and facilitate diagnosis and treatment. Most patients also need additional treatment for the underlying psychiatric condition.

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

PubMed

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.

Corticalization of motor control in humans is a consequence of brain scaling in primate evolution.

PubMed

Herculano-Houzel, Suzana; Kaas, Jon H; de Oliveira-Souza, Ricardo

2016-02-15

Control over spinal and brainstem somatomotor neurons is exerted by two sets of descending fibers, corticospinal/pyramidal and extrapyramidal. Although in nonhuman primates the effect of bilateral pyramidal lesions is mostly limited to an impairment of the independent use of digits in skilled manual actions, similar injuries in humans result in the locked-in syndrome, a state of mutism and quadriplegia in which communication can be established only by residual vertical eye movements. This behavioral contrast makes humans appear to be outliers compared with other primates because of our almost total dependence on the corticospinal/pyramidal system for the effectuation of movement. Here we propose, instead, that an increasing preponderance of the corticospinal/pyramidal system over motor control is an expected consequence of increasing brain size in primates because of the faster scaling of the number of neurons in the primary motor cortex over the brainstem and spinal cord motor neuron pools, explaining the apparent uniqueness of the corticalization of motor control in humans. © 2015 Wiley Periodicals, Inc.

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

PubMed Central

Assareh, Marzieh

2010-01-01

Neuroleptic 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. PMID:22952496

Encephalitis Lethargica With Isolated Substantia Nigra Lesions Followed by a Second Encephalitis in a Child With Humoral Immunodeficiency.

PubMed

Yang, Lu; Jia, Guijuan; Li, Baomin; Lei, Gefei; Sun, Ruopeng

2015-12-01

Encephalitis lethargica is an encephalitic illness with multiple nervous system symptoms. Lesions only involving substantia nigra on magnetic resonance imaging are uncommon, especially in children. A second encephalitis illness after encephalitis lethargica has never been reported before. We describe a 7-year-old boy with humoral immunity deficiency who developed encephalitis lethargica associated with bilateral substantia nigra lesions on magnetic resonance imaging. After a nearly complete recovery, he developed encephalitis once again. He was diagnosed with encephalitis lethargica with somnolence, akinetic mutism, and ophthalmoplegia after intermittent fever. Cerebrospinal fluid pleocytosis and positive oligoclonal bands were documented. Symmetrical substantia nigra lesions on high-intensity magnetic resonance imaging gradually evolved into a liquid signal. He had almost recovered when he developed fatigue and hypersomnia and was diagnosed with encephalitis again, supported by mild pleocytosis in cerebrospinal fluid and subcortical white matter lesions in the frontal lobes. His symptoms resolved following administration of corticosteroids and immunoglobulins. This is the first report of an immune-deficient child to develop encephalitis lethargica with isolated substantia nigra lesions on magnetic resonance imaging and a second encephalitis illness after recovery from encephalitis lethargica. Copyright © 2015. Published by Elsevier Inc.

[A psycholinguistic study in a patient with echolalia].

PubMed

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.

Effective treatment of catatonia by combination of benzodiazepine and electroconvulsive therapy.

PubMed

Unal, Ahmet; Bulbul, Feridun; Alpak, Gokay; Virit, Osman; Copoglu, U Sertan; Savas, Haluk A

2013-09-01

Catatonia, a motor dysregulation syndrome, can emerge in numerous psychiatric disorders, mainly in schizophrenia and mood disorders, and metabolic and endocrine disorders such as infections, toxic states, epilepsy, and traumatic brain injury. In our study, we aimed to investigate demographic, clinical, and treatment-related characteristics of catatonic patients managed in our inpatient clinic. The medical records of 57 patients diagnosed to have catatonia according to the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision, criteria who were admitted to the inpatient psychiatry clinic of the Gaziantep University School of Medicine between 1 January, 2003, and 31 December, 2011, were retrospectively reviewed. In patients with catatonia, mood disorders (63.2%) were found to be the most common underlying or primary disease, whereas mutism (47.4%) was found to be the most common catatonic symptom. There was a comorbid medical condition in 9 patients (15.8%). Patients underwent an average of 9.00 electroconvulsive therapy (ECT) sessions. Among 57 patients with catatonia, catatonic symptoms were resolved in 57 patients (100%) by benzodiazepine and ECT. In our study, full recovery was achieved in catatonia by benzodiazepine plus ECT combination. As a result, we recommend combined ECT and benzodiazepine for catatonia.

Three cases of Creutzfeldt-Jakob disease with prion protein gene codon180 mutation presenting with pathological laughing and crying.

PubMed

Iwasaki, Yasushi

2012-08-15

Although there are no reports of pathological laughing and crying being observed in patients with Creutzfeldt-Jakob disease (CJD), the author experienced three patients with CJD with prion protein gene codon180 mutation (V180I CJD) who showed this characteristic clinical finding. This finding was observed from the early disease stage in all 3 patients and continued for several months. Startle reaction was also remarkable in all patients, although myoclonus was generally mild. The dissociation between the startle reaction and myoclonus was suspected to be another feature of V180I CJD. The pathological laughing and crying co-occured with the startle reaction and stopped right before the onset of akinetic mutism, and the degree of both symptoms was almost parallel during this period. On the basis of MRI and autopsy findings, pathological laughing and crying was suspected of being induced by the widespread cerebral cortical involvement that is characteristic of V180I CJD. From the present observations, the author speculated that pathological laughing and crying may be a comparatively frequent observation in V180I CJD patients. Copyright © 2012 Elsevier B.V. All rights reserved.

Subacute sclerosing panencephalitis: an update.

PubMed

Gutierrez, Jose; Issacson, Richard S; Koppel, Barbara S

2010-10-01

Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis occurring after infection with measles virus. The prevalence of the disease varies depending on uptake of measles vaccination, with the virus disproportionally affecting regions with low vaccination rates. The physiopathology of the disease is not fully understood; however, there is evidence that it involves factors that favour humoral over cellular immune response against the virus. As a result, the virus is able to infect the neurons and to survive in a latent form for years. The clinical manifestations occur, on average, 6 years after measles virus infection. The onset of SSPE is insidious, and psychiatric manifestations are prominent. Subsequently, myoclonic seizures usually lead to a final stage of akinetic mutism. The diagnosis is clinical, supported by periodic complexes on electroencephalography, brain imaging suggestive of demyelination, and immunological evidence of measles infection. Management of the disease includes seizure control and avoidance of secondary complications associated with the progressive disability. Trials of treatment with interferon, ribavirin, and isoprinosine using different methodologies have reported beneficial results. However, the disease shows relentless progression; only 5% of individuals with SSPE undergo spontaneous remission, with the remaining 95% dying within 5 years of diagnosis.

Neuronal Control of Mammalian Vocalization, with Special Reference to the Squirrel Monkey

NASA Astrophysics Data System (ADS)

Jürgens, Uwe

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

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

PubMed Central

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

Long-term functional outcome of patients with cerebellar pilocytic astrocytoma surgically treated in childhood.

PubMed

Ait Khelifa-Gallois, N; Laroussinie, F; Puget, S; Sainte-Rose, C; Dellatolas, G

2015-01-01

Abstract Purpose: A number of studies report neurological and cognitive deficits and behavioural disorders in children after surgical treatment for a benign cerebellar tumour. The present study explores functional outcome in adolescents and adults treated for a low-grade cerebellar astrocytoma in childhood. Participants were 18 adolescents and 46 adults treated for low-grade astrocytoma in childhood. Academic achievement, professional status and neurological, cognitive and behavioural disturbances were collected using self-completed and parental questionnaires for adolescents and phone interview for adults. For the adolescent group, a control group filled in the same questionnaires. Mean time lapse from surgery was 7.8 years for adolescents and 12.9 years for adults. Five adults (11%) had major sequelae related to post-operative complications, post-operative mutism and/or brain stem involvement. All the other participants presented close-to-normal academic achievement and normal autonomy, despite a high rate of reported cognitive difficulties and difficulties related to mild neurological sequelae (fine motor skills, balance). The long-term functional outcome of low-grade cerebellar astrocytoma is generally favourable, in the absence of post-operative complications and brain stem involvement. No major impact of neurological deficits, cognitive problems and emotional disorders on academic achievement and independent functioning was observed.

Rediscovering catatonia: the biography of a treatable syndrome.

PubMed

Fink, Max

2013-01-01

Catatonia, a disorder of movement and mood, was described and named in 1874. Other observers quickly made the same recognition. By the turn of the century, however, catatonia was incorporated as a type within a conjured syndrome of schizophrenia. There, catatonia has lain in the psychiatric classification for more than a century. We review the history of catatonia and its present status. In the 1970s, the tie was questioned when catatonia was recognized among those with mood disorders. The recognition of catatonia within the neuroleptic malignant syndrome offered effective treatments of high doses of benzodiazepines and electroconvulsive therapy (ECT), again questioning the tie. A verifying test for catatonia (the lorazepam sedation test) was developed. Soon the syndromes of delirious mania, toxic serotonin syndrome, and the repetitive behaviors in adolescents with autism were recognized as treatable variations of catatonia. Ongoing studies now recognize catatonia among patients labeled as suffering from the Gilles de la Tourette's syndrome, anti-NMDAR encephalitis, obsessive-compulsive disease, and various mutisms. Applying the treatments for catatonia to patients with these syndromes offers opportunities for clinical relief. Catatonia is a recognizable and effectively treatable neuropsychiatric syndrome. It has many faces. It warrants recognition outside schizophrenia in the psychiatric disease classification. © 2012 John Wiley & Sons A/S. Published by Blackwell Publishing Ltd.

[Opsoclonus-myoclonus syndrome in a 2 year old boy with prenatally diagnosed retroperitoneal tumour].

PubMed

Jamroz, Ewa; Głuszkiewicz, Ewa; Madziara, Wojciech; Kiełtyka, Aleksandra

2011-01-01

Opsoclonus-myoclonus syndrome, also named Myoclonic Encephalopathy of Infants, Opsoclonus- Myoclonus Ataxia, Dancing Eyes - Dancing Feet Syndrome, Dancing Eyes Syndrome, Kinsbourne syndrome, is a rare, paraneoplastic or possibly post-viral chronic neurological disorder. The age of presentation ranges from 6 months to 3 years. In 50% of affected children the syndrome is associated with an underlying occult or clinically apparent neuroblastoma. In most patients the tumour is localized, small and well differentiated, with no NMYC gene copy number amplification. The syndrome may also occur after tumour resection or at relapse. The opsoclonus-myoclonus syndrome can occur in children without neuroblastoma, in such idiopathiccases, the onset of neurological symptoms is related to infection. It is assumed, that in idiopathic cases the syndrome could have developed in the course of neuroblastoma which had undergone a complete spontaneous regression. The most characteristic clinical features of opsoclonus-myoclonus syndrome are: opsoclonus, myoclonus, ataxia, irritability, mutism and sleep disturbances. The disease course is usually long-term with episodes of remission and relapses. Approximately 80% of children with opsoclonus-myoclonus syndrome suffer from mild to severe neurological handicaps, mainly cognitive impairment. The authors present a 2-year old boy with opsoclonus-myoclonus syndrome preceded by involution of prenatally documented retroperitoneal area tumour.

[Recovery of consciousness: process-oriented approach].

PubMed

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.

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

PubMed Central

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

A misdiagnosed patient: 16 years of locked-in syndrome, the influence of rehabilitation.

PubMed

Lukowicz, Malgorzata; Matuszak, Katarzyna; Talar, Anna

2010-02-01

LiS can be mistakenly recognized as a vegetative state, minimally conscious state or akinetic mutism. It can be caused by isolated lesions--bilateral infarction, vertebrobasilar artery osslusion, haemorrhage or tumor of the ventral portion of the basis pontis or midbrain. The case of a 65-year-old patient with a brain tumor localized in the posterior part of the posterior commissure of the brain was presented. He lost consciousness in 1991, was diagnosed as being at a terminal stage and from 2005 he started to improve. In MRI brain tumor stated in 1989 with the same localization and size in 2007 without any disturbance in cerebral fluid flow. The patients remained in this condition for 14 years without any rehabilitation, because he was diagnosed as a terminal stage, a non-operative stage. When exercises were introduced in 2005 the patient started to recover. In 2007 he was conscious with quadriplegia, a neuropsychological test showed memory problems, without any dementia. After intensive rehabilitation functional improvement and speech improvement was observed, GOS (4), Ranczo Los Amigos Scale (6), DRS (18). It is important to carry out full diagnostics before determining a terminal stage and to continue a rehabilitation program and multisensory stimulation. Even after 16 year of lying in bed without communication there is a chance in LiS to witness improvement after stimulation, without any signs of dementia.

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

PubMed

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.

[German version of the Northoff catatonia rating scale (NCRS-dv) : A validated instrument for measuring catatonic symptoms].

PubMed

Hirjak, D; Thomann, P A; Northoff, G; Kubera, K M; Wolf, R C

2017-07-01

The clinical picture of catatonia includes impressive motor phenomena, such as rigidity, dyskinesia, festination, negativism, posturing, catalepsy, stereotypies and mannerisms, along with affective (e. g. aggression, anxiety, anhedonism or emotional lability) and behavioral symptoms (e.g. mutism, autism, excitement, echolalia or echopraxia). In English speaking countries seven catatonia rating scales have been introduced, which are widely used in clinical and scientific practice. In contrast, only one validated catatonia rating scale is available in Germany so far. In this paper, we introduce the German version of the Northoff catatonia rating scale (NCRS-dv). The original English version of the NCRS consists of 40 items describing motor (13 items), affective (12 items) and behavioral (15 items) catatonic symptoms. The NCRS shows high internal reliability (Crombachs alpha = 0.87), high interrater (r = 0.80-0.96) and high intrarater (r = 0.80-0.95) reliability. Factor analysis of the NCRS revealed four domains: affective, hyperactive or excited, hypoactive or retarded and behavior with individual eigenvalues of 8.98, 3.61, 2.98 and 2.82, respectively, which explained 21.5 %, 9.3 %, 7.6 % and 7.2 % of variance, respectively. In conclusion, the NCRS-dv represents a second validated instrument which can be used by German clinicians and scientists for the assessment of catatonic symptoms.

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

PubMed Central

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

2016-01-01

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

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

PubMed

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

2016-09-01

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.

The contribution of the cerebellum to speech production and speech perception: clinical and functional imaging data.

PubMed

Ackermann, Hermann; Mathiak, Klaus; Riecker, Axel

2007-01-01

A classical tenet of clinical neurology proposes that cerebellar disorders may give rise to speech motor disorders (ataxic dysarthria), but spare perceptual and cognitive aspects of verbal communication. During the past two decades, however, a variety of higher-order deficits of speech production, e.g., more or less exclusive agrammatism, amnesic or transcortical motor aphasia, have been noted in patients with vascular cerebellar lesions, and transient mutism following resection of posterior fossa tumors in children may develop into similar constellations. Perfusion studies provided evidence for cerebello-cerebral diaschisis as a possible pathomechanism in these instances. Tight functional connectivity between the language-dominant frontal lobe and the contralateral cerebellar hemisphere represents a prerequisite of such long-distance effects. Recent functional imaging data point at a contribution of the right cerebellar hemisphere, concomitant with language-dominant dorsolateral and medial frontal areas, to the temporal organization of a prearticulatory verbal code ('inner speech'), in terms of the sequencing of syllable strings at a speaker's habitual speech rate. Besides motor control, this network also appears to be engaged in executive functions, e.g., subvocal rehearsal mechanisms of verbal working memory, and seems to be recruited during distinct speech perception tasks. Taken together, thus, a prearticulatory verbal code bound to reciprocal right cerebellar/left frontal interactions might represent a common platform for a variety of cerebellar engagements in cognitive functions. The distinct computational operation provided by cerebellar structures within this framework appears to be the concatenation of syllable strings into coarticulated sequences.

[Clinical heterogeneity of Alzheimer's disease. Different clinical profiles can predict the progression rate].

PubMed

Mangone, C A

Alzheimer's disease (AD) is a degenerative dementia that may disclose different cognitive, behavioral, psychiatric and functional symptoms since onset. These distinct cognitive profiles support the conception of clinical heterogeneity and account for AD's highly variable rate of progression. In spite of strict diagnostic criteria NINCS ADRDA's and DSM IV the clinical certainty is only about 85%. Mayeux define 4 subtypes: a). Benign: mild cognitive and functional impairment without focal signs and late onset behavioral signs, slow progression; b). Myoclonic: usually of presenile onset with severe cognitive deterioration, mutism and early onset myoclonus; c). Extrapyramidal: early onset akineto rigid signs with severe cognitive, behavioral and psychiatric involvement; d). Typical: gradual and progressive cognitive, behavioral and functional impairment. The differentiation of these subtypes will allow us to define discrete patterns of progression, to define prognostic subgroups, and to homogenize them for clinical research and drug trials. We examined 1000 charts of probable AD patients from the Santojanni Center. We found 42% extrapyramidal, 35% typical, 15% benign and 8% myoclonic. The early onset of parkinsonism and myoclonus predict a rapidly evolving cognitive impairment and a more severe rate of progression with psychiatric disorders and dependency in activities of daily living. (DADL) Patients with low level of education, low cognitive performance at entry as well as those with rapid rate of cognitive deterioration had a faster rate of progression to DADL. Delusions, low level of education, extrapyramidal signs and motor hyperactivity but not hallucinations, and anosognosia were the best non cognitive predictors of DADL.

Neuropsychiatric phenomena in the medieval text Cantigas de Santa Maria.

PubMed

Gondim, Francisco De Assis Aquino; Griesbach, Sarah H; Thomas, Florian P

2015-05-12

To discuss the neuropsychiatric phenomena described in Cantigas de Santa Maria (Canticles of St. Mary [CSM]). CSM is a collection of 427 canticles composed in Galician-Portuguese between 1252 and 1284 at the Court of King Alfonso X the Wise of Spain (1221-1284). The canticles (of which 9 are repeated) include devotional and liturgical poems and 353 narrative stories consisting mainly of depictions of Marian miracles. Most are set to music and many are illustrated. We reviewed the canticles for description of miracles and other neuropsychiatric phenomena. Two neurologists reached a consensus about the descriptions. Of the 353 miracles, 279 medically relevant facts (from 187 canticles) and 25 instances of resurrection were reported. Possible neuropsychiatric conditions were described in 98 canticles. Physicians were mentioned in 16 narratives. The most common neurologic conditions detailed were blindness (n = 17), dystonia, weakness, and deformities (n = 20). Other common conditions included psychosis (n = 15), speech disorder/deaf-mutism (n = 12), infections (n = 15), sexual dysfunction/infertility/obstetrical-gynecologic issues (n = 18), head trauma (n = 5), ergotism/St. Anthony's fire (n = 7), and others. There were 9 instances of prodromic mystical experiences/hallucinations heralding death. While limited by retrospection and interpretation of neuropsychiatric phenomena in the medieval context, these short accounts are among the first descriptions of neuropsychiatric conditions in early Portuguese/Galician. They reflect how medieval societies used rational and irrational approaches to understand occurrences in their lives. © 2015 American Academy of Neurology.

Gerstmann-Straeussler-Scheinker disease with P102L prion protein gene mutation presenting with rapidly progressive clinical course.

PubMed

Iwasaki, Yasushi; Mori, Keiko; Ito, Masumi; Nokura, Kazuya; Tatsumi, Shinsui; Mimuro, Maya; Kitamoto, Tetsuyuki; Yoshida, Mari

2014-01-01

We describe an autopsied case of a Japanese woman with Gerstmann-Straeussler-Scheinker disease (GSS) presenting with a rapidly progressive clinical course. Disease onset occurred at the age of 54 with dementia and gait disturbance. Her clinical course progressively deteriorated until she reached a bedridden state with myoclonus 9 months after onset. Two months later, she reached the akinetic mutism state. Nasal tube feeding was introduced at this point and continued for several years. Electroencephalograms showed diffuse slowing without periodic sharp-wave complexes. Diffusion-weighted magnetic resonance imaging (MRI) showed widespread cerebral cortical hyperintensity. Prion protein (PrP) gene analysis revealed a Pro to Leu point mutation at codon 102 with methionine homozygosity at codon 129. The patient died of respiratory failure after a total disease duration of 62 months. Neuropathologic examination revealed widespread spongiform change with numerous eosinophilic amyloid plaques (Kuru plaques) in the cerebral and cerebellar cortices by H & E staining. Diffuse myelin pallor with axon loss of the cerebral white matter, suggestive of panencephalopathic-type pathology was observed. Numerous PrP immunopositive plaques and diffuse synaptic-type PrP deposition were extensively observed, particularly in the cerebral and cerebellar cortices. Western blot analysis of proteinase Kresistant PrP showed a characteristic band pattern with a small molecular band of 6 kDa. The reason for the similarity in clinicopathologic findings between the present case and Creutzfeldt-Jakob disease is uncertain; however, the existence of an unknown disease-modifying factor is suspected.

Catatonia in Resource Limited Settings: A Case Series and Treatment Protocol

PubMed Central

Smith, Stephanie L.; Grelotti, David J.; Fils-Aime, Reginald; Uwimana, Eugenie; Ndikubwimana, Jean-Sauveur; Therosme, Tatiana; Severe, Jennifer; Dushimiyimana, Dominique; Uwamariya, Clemence; Bienvenu, Robert; Alcindor, Yoldie; Eustache, Eddy; Raviola, Giuseppe J.; Fricchione, Gregory L.

2014-01-01

Objective The Catatonic Syndrome (“catatonia”) is characterized by motor and motivation dysregulation and is associated with a number of neuropsychiatric and medical disorders. It is recognizable in a variety of clinical settings. We present observations from the treatment of four individuals with catatonia in Haiti and Rwanda, and introduce a treatment protocol for use in resource limited settings Methods Four patients from rural Haiti and Rwanda with clinical signs of catatonia and a positive screen using the Bush-Francis-Catatonia Rating Scale were treated collaboratively by general physicians and mental health clinicians with either lorazepam or diazepam. Success in treatment was clinically assessed by complete remittance of catatonia symptoms. Results The four patients in this report exhibited a range of characteristic and recognizable signs of catatonia, including immobility/stupor, stereotypic movements, echophenomena, posturing, odd mannerisms, mutism, and refusal to eat or drink. All four cases presented initially to rural outpatient general health services in low resource settings. In some cases, diagnostic uncertainty initially led to treatment with typical antipsychotics. In each case, proper identification and treatment of catatonia with benzodiazepines led to significant clinical improvement. Conclusion Catatonia can be effectively and inexpensively treated in resource limited settings. Identification and management of catatonia is critical for the health and safety of patients with this syndrome. Familiarity with the clinical features of catatonia is essential for health professionals working in low resource settings. To facilitate early recognition of this treatable disorder, catatonia should feature more prominently in global mental health discourse. PMID:25467078

[A case of Asperger's disorder with catatonia originally suspected of being catatonic schizophrenia].

PubMed

Saito, Shinnosuke; Yamaga, Kuniaki; Kobayashi, Toshiyuki; Kato, Satoshi

2011-01-01

We report the case of an adolescent male who presented with mutism, immobility, catalepsy, and mannerisms. The patient was admitted to our hospital with suspected catatonic schizophrenia; however, he was subsequently diagnosed with catatonia due to Asperger's disorder. The patient was a 16-year-old male. More than six months before presentation, his grandfather displayed bizarre and violent behavior. Subsequently, he began to experience catatonia, which eventually led to hospitalization. Treatment with diazepam improved his condition and, as no causal disorders other than Asperger's disorder were identified, he was diagnosed with catatonia. The patient had experienced persistent abuse by his mother during childhood; therefore, it is important to consider reactive attachment disorder (DSM-IV-TR) as a differential diagnosis. Among child and adolescent psychiatrists, catatonia is considered to occur at a high frequency among patients with autistic spectrum disorders. In contrast, general psychiatrists tend to consider catatonia as related to schizophrenia, which may be the reason why the diagnosis of our patient was difficult. We assume that the pathogenesis of catatonia in this case was death mimicry due to the subjective perception of a life-threatening situation. For the treatment of catatonia with autistic spectrum disorders, the efficacy of benzodiazepines and electroconvulsive therapy has been established. When a patient with an autistic spectrum disorder presents with motor functional disturbances, it is important to consider these disturbances as catatonia. Furthermore, it is also important to begin the treatment mentioned above even in the presence of definite psychogenic or situational factors.

Catatonia After Cerebral Hypoxia: Do the Usual Treatments Apply?

PubMed Central

Quinn, Davin K.; Abbott, Christopher C.

2014-01-01

Introduction Neurologic deterioration occurring days to weeks after a cerebral hypoxic event accompanied by diffuse white matter demyelination is called delayed post-hypoxic leukoencephalopathy (DPHL). Manifestations of DPHL are diverse, and include dementia, gait disturbance, incontinence, pyramidal tract signs, parkinsonism, chorea, mood and thought disorders, akinetic mutism, and rarely catatonia. Methods The authors report a case of malignant catatonia in a patient diagnosed with DPHL that was refractory to electroconvulsive therapy (ECT), and review the literature on catatonia in DHPL. Results The patient was a 56 year-old female with schizoaffective disorder who was admitted with catatonia two weeks after hospitalization for drug overdose and respiratory failure. Her catatonic symptoms did not respond to lorazepam, amantadine, methylphenidate, or ten sessions of bilateral ECT at maximum energy. Repeat magnetic resonance imaging revealed extensive periventricular white matter lesions not present on admission scans, and she was diagnosed with DPHL. Discussion No treatment for DPHL has been proven to be widely effective. Hyperbaric oxygen treatments may reduce the rate of development, and symptom improvement has been reported with stimulants and other psychotropic agents. Review of the literature reveals rare success with GABAergic agents for catatonia after cerebral hypoxia, and no cases successfully treated with ECT. There are seven case reports of neurologic decompensation during ECT treatment after a cerebral hypoxic event. Conclusion Caution is advised when considering ECT for catatonia when delayed sequelae of cerebral hypoxia are on the differential diagnosis, as there is a dearth of evidence to support this treatment approach. PMID:25262046

[Bilateral caudate head infarcts].

PubMed

Kuriyama, N; Yamamoto, Y; Akiguchi, I; Oiwa, K; Nakajima, K

1997-11-01

We reported a 67-year-old woman with bilateral caudate head infarcts. She developed sudden mutism followed by abulia. She was admitted to our hospital 2 months after ictus for further examination. She showed prominent abulia and was inactive, slow and apathetic. Spontaneous activity and speech, immediate response to queries, spontaneous word recall and attention and persistence to complex programs were disturbed. Apparent motor disturbance, gait disturbance, motor aphasia, apraxia and remote memory disturbance were not identified. She seemed to be depressed but not sad. Brain CT and MRI revealed bilateral caudate head hemorrhagic infarcts including bilateral anterior internal capsules, in which the left lesion was more extensive than right one and involved the part of the left putamen. These infarct locations were thought to be supplied by the area around the medial striate artery including Heubner's arteries and the A1 perforator. Digital subtraction angiography showed asymptomatic right internal carotid artery occlusion. She bad had hypertension, diabetes mellitus and atrial fibrillation and also had a left atrium with a large diameter. The infarcts were thought to be caused by cardioembolic occlusion to the distal portion of the left internal carotid artery. Although some variations of vasculature at the anterior communicating artery might contribute to bilateral medial striate artery infarcts, we could not demonstrate such abnormalities by angiography. Bilateral caudate head infarcts involving the anterior internal capsule may cause prominent abulia. The patient did not improve by drug and rehabilitation therapy and died suddenly a year after discharge.

Consciousness, information integration, and the brain.

PubMed

Tononi, Giulio

2005-01-01

Clinical observations have established that certain parts of the brain are essential for consciousness whereas other parts are not. For example, different areas of the cerebral cortex contribute different modalities and submodalities of consciousness, whereas the cerebellum does not, despite having even more neurons. It is also well established that consciousness depends on the way the brain functions. For example, consciousness is much reduced during slow wave sleep and generalized seizures, even though the levels of neural activity are comparable or higher than in wakefulness. To understand why this is so, empirical observations on the neural correlates of consciousness need to be complemented by a principled theoretical approach. Otherwise, it is unlikely that we could ever establish to what extent consciousness is present in neurological conditions such as akinetic mutism, psychomotor seizures, or sleepwalking, and to what extent it is present in newborn babies and animals. A principled approach is provided by the information integration theory of consciousness. This theory claims that consciousness corresponds to a system's capacity to integrate information, and proposes a way to measure such capacity. The information integration theory can account for several neurobiological observations concerning consciousness, including: (i) the association of consciousness with certain neural systems rather than with others; (ii) the fact that neural processes underlying consciousness can influence or be influenced by neural processes that remain unconscious; (iii) the reduction of consciousness during dreamless sleep and generalized seizures; and (iv) the time requirements on neural interactions that support consciousness.

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

PubMed

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.

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

PubMed

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.

Speech disorders of Parkinsonism: a review.

PubMed Central

Critchley, E M

1981-01-01

Study of the speech disorders of Parkinsonism provides a paradigm of the integration of phonation, articulation and language in the production of speech. The initial defect in the untreated patient is a failure to control respiration for the purpose of speech and there follows a forward progression of articulatory symptoms involving larynx, pharynx, tongue and finally lips. There is evidence that the integration of speech production is organised asymmetrically at thalamic level. Experimental or therapeutic lesions in the region of the inferior medial portion of ventro-lateral thalamus may influence the initiation, respiratory control, rate and prosody of speech. Higher language functions may also be involved in thalamic integration: different forms of anomia are reported with pulvinar and ventrolateral thalamic lesions and transient aphasia may follow stereotaxis. The results of treatment with levodopa indicates that neurotransmitter substances enhance the clarity, volume and persistence of phonation and the latency and smoothness of articulation. The improvement of speech performance is not necessarily in phase with locomotor changes. The dose-related dyskinetic effects of levodopa, which appear to have a physiological basis in observations previously made in post-encephalitic Parkinsonism, not only influence the prosody of speech with near-mutism, hesitancy and dysfluency but may affect work-finding ability and in instances of excitement (erethism) even involve the association of long-term memory with speech. In future, neurologists will need to examine more closely the role of neurotransmitters in speech production and formulation. PMID:7031185

Paraneoplastic neurological disorders in children with benign ovarian tumors.

PubMed

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.

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

NASA Astrophysics Data System (ADS)

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 Phenomenology and Treatment Response in Catatonia: A Hospital Based Descriptive Study

PubMed Central

Swain, Sarada Prasanna; Behura, Sushree Sangita; Dash, Manoj Kumar

2017-01-01

Background: Literatures regarding clinical symptomatology and treatment response of catatonia are very few. Objective: To assess onset, clinical profile, diagnostic break up, treatment response and outcome in patients diagnosed as Catatonia, reported to a tertiary care hospital. Methods: The present study was a cross-sectional descriptive study conducted in indoor of Mental Health Institute (Centre of Excellence), S.C.B. Medical College, between March 2015 to March 2016. A total of 34 patients were included in the study who reported at outdoor department of Mental Health Institute with catatonic symptoms. All patients admitted in inpatient department were routinely assessed through a detailed semi-structured interview. The diagnosis of catatonia was made if the patients present with three or more symptoms out of twelve symptoms fulfilling the criteria of DSM-5. All the patients were assessed through Bush-Francis Catatonia Rating Scale. They were initially given parental lorazepam at the doses ranging from 4-12 mg per day as per requirement. Patients who did not respond to lorazepam trial were given ECT. Results: The patients were predominantly presented with retarded symptoms of catatonia such as staring, mutism, withdrawal, posturing and negativism. Schizophrenia and other psychotic spectrum disorders were more commonly presented as catatonia as compared to mood disorders. Younger age group patients were mainly responded to lorazepam only, whereas older age group patients responded to both ECT and lorazepam. Conclusion: This study has came out with very important insights in the age of incidence, phenomenology, clinical profile, source of referral, diagnostic break up and treatment response with lorazepam and ECT in catatonic patients following mental disorder. PMID:28615768

Colloid cysts of the third ventricle. Endoscopic and open microsurgical management.

PubMed

Stachura, Krzysztof; Libionka, Witold; Moskała, Marek; Krupa, Mariusz; Polak, Jarosław

2009-01-01

The endoscopic approach to colloid cysts of the third ventricle is receiving increasing interest. However, its effectiveness is a matter of discussion. The aim of the study was to present direct and long-term outcome after endoscopy of colloid cyst vs microsurgery. Medical records of 23 patients with colloid cysts were retrospectively analyzed. This group consists of 10 patients treated endoscopically and 13 patients treated using a transcortical-transventricular approach. Sex and age distributions were similar in both groups. Clinically, symptoms of raised intracranial pressure predominated. All patients had hydrocephalus. Tumour diameter ranged from 1.5 to 3 cm. Mean follow-up period was 31 months. In 6/10 endoscopically treated patients, tumours were completely removed. In 3 patients, small capsule remnants, adherent to the choroid plexus and veins, were left. In one case, a portion of capsule, obstructing the intraventricular foramen, was finally removed microsurgically. Postoperatively, 2 patients complained of memory deficits, which became permanent in one case. One patient developed temporary mutism. In one case, with symptoms of hydrocephalus without colloid cyst recurrence, a ventriculoperitoneal shunt was implanted 6 months after the initial surgery. In all microsurgically treated patients tumours were completely removed. One patient was reoperated because of intracerebral haematoma. Two patients suffered from temporary hemiparesis and 2 developed epilepsy. Within one year after surgery 3 patients were shunted because of hydrocephalus; one patient required antiepileptic treatment. The endoscopic approach to colloid cysts of the third ventricle is safe, effective and carries a low complication rate. Endoscopy may be recommended as a treatment option.

Surgical and clinical aspects of cerebellar pilomyxoid-spectrum astrocytomas in children.

PubMed

El Beltagy, Mohamed A; Atteya, Mostafa M E; El-Haddad, Alaa; Awad, Madiha; Taha, Hala; Kamal, Mohamed; El Naga, Sherif Abou

2014-06-01

Cerebellar pilomyxoid astrocytomas (PMAs) and intermediate pilomyxoid astrocytomas (IPAs) are collectively called "pilomyxoid-spectrum astrocytomas (PMSAs)." Cerebellar PMSAs are thought to behave more aggressively than pilocytic astrocytomas (PAs). Our objective is to compare PMSAs to PAs in terms of surgical and clinical profiles. This retrospective study included 66 cases (35 males and 31 females) with cerebellar astrocytomas treated between July 2007 and December 2012 at Children's Cancer Hospital Egypt (CCHE 57357) with a mean age of 7 (±1.5) years. Cases were divided into three subgroups as follows: 44 PAs, 10 IPAs, and 12 PMAs. Comparison between all groups was focusing on brain stem invasion, intrinsic necrotic cavitation, extent of resection, recurrence, leptomeningeal dissemination (LD), metastases, need for CSF diversion, and cerebellar mutism (CM). Cerebellar PMAs and IPAs separately and collectively had higher incidence of brain stem invasion, intrinsic necrotic cavitation, tumor recurrence, and LD when compared to PAs (P < 0.001). Gross total resection was 13.6 % in PMSAs versus 90.9 % in PAs (P < 0.001). PMAs had a higher incidence of tumor recurrence than IPAs (66.7 versus 20 %, P < 0.001). Incidence of recurrence in PAs was 9.1 % in partially resected cases. Mean interval to recurrence was 9 (±1.5) months in PMSAs and 42 (±2) months in PAs. Cerebellar PMSAs express an aggressive clinical behavior and impose more operative challenges than PAs. These tumors may represent a clinical spectrum-at its benign end lies PA, while PMA lies at the aggressive end, with IPA lying just behind. Such concepts could be used to guide management in the future.

Proximal dentatothalamocortical tract involvement in posterior fossa syndrome

PubMed Central

Phillips, Nicholas S.; Laningham, Fred H.; Patay, Zoltan; Gajjar, Amar; Wallace, Dana; Boop, Frederick; Sanford, Robert; Ness, Kirsten K.; Ogg, Robert J.

2009-01-01

Posterior fossa syndrome is characterized by cerebellar dysfunction, oromotor/oculomotor apraxia, emotional lability and mutism in patients after infratentorial injury. The underlying neuroanatomical substrates of posterior fossa syndrome are unknown, but dentatothalamocortical tracts have been implicated. We used pre- and postoperative neuroimaging to investigate proximal dentatothalamocortical tract involvement in childhood embryonal brain tumour patients who developed posterior fossa syndrome following tumour resection. Diagnostic imaging from a cohort of 26 paediatric patients previously operated on for an embryonal brain tumour (13 patients prospectively diagnosed with posterior fossa syndrome, and 13 non-affected patients) were evaluated. Preoperative magnetic resonance imaging was used to define relevant tumour features, including two potentially predictive measures. Postoperative magnetic resonance and diffusion tensor imaging were used to characterize operative injury and tract-based differences in anisotropy of water diffusion. In patients who developed posterior fossa syndrome, initial tumour resided higher in the 4th ventricle (P = 0.035). Postoperative magnetic resonance signal abnormalities within the superior cerebellar peduncles and midbrain were observed more often in patients with posterior fossa syndrome (P = 0.030 and 0.003, respectively). The fractional anisotropy of water was lower in the bilateral superior cerebellar peduncles, in the bilateral fornices, white matter region proximate to the right angular gyrus (Tailerach coordinates 35, –71, 19) and white matter region proximate to the left superior frontal gyrus (Tailerach coordinates –24, 57, 20). Our findings suggest that multiple bilateral injuries to the proximal dentatothalamocortical pathways may predispose the development of posterior fossa syndrome, that functional disruption of the white matter bundles containing efferent axons within the superior cerebellar peduncles is a critical underlying pathophysiological component of posterior fossa syndrome, and that decreased fractional anisotropy in the fornices and cerebral cortex may be related to the abnormal neurobehavioural symptoms of posterior fossa syndrome. PMID:19805491

Children with 7q11.23 Duplication Syndrome: Psychological Characteristics

PubMed Central

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

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

PubMed Central

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 potential solutions, including modular treatment formats and innovative applications of technology. PMID:25212716

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

PubMed Central

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

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

PubMed

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.

Clinical and epidemiological aspects of methylmercury poisoning.

PubMed Central

Bakir, F.; Rustam, H.; Tikriti, S.; Al-Damluji, S. F.; Shihristani, H.

1980-01-01

An opportunity to study the effects of methylmercury poisoning in humans was provided by the large outbreak in Iraq in 1971-2. In adults, poisoning resulted from the ingestion of home-made bread prepared from methylmercury-treated seed grain and there was a highly significant correlation between the amount of bread ingested and blood mercury levels. Poisoning in infants resulted either from prior exposure in utero or from suckling or both. Blood mercury levels were higher in infants and children than in adults. There was no increased incidence of congenital defects. Symptoms and signs of poisoning and histopathological changes were mainly confined to the CNS. Symptoms developed, on average, 1-2 months after exposure. In children there was mental retardation with delayed onset of speech and impaired motor, sensory and autonomic function. Severely affected children were blind and deaf. In adults, the clinical picture could be classified as 1, mild (mainly of sensory symptoms) 2, moderate (sensory symptoms accompanied by cerebellar signs) and 3, severe (gross ataxia with marked visual and hearing loss which, in some cases, progressed to akinetic mutism followed by coma). Grades 1 and 2 carried a better prognosis thant grade 3. Interference with transmission at the myoneural junction was found in 14% of patients studied. There was no evidence of peripheral nerve involvement per se and sensory symptoms may be of central origin. The clinical differences between the Iraqi and Japanese outbreaks may be a result, in part at least, of the severe, prolonged and continuous exposure which occurred in the latter outbreak. Improvement was observed among the mild and moderate group. Treatment with chelating agents, thiol resin, haemodialysis and exchange transfusion lowered blood mercury concentrations but produced no convincing clinical benefit. To be effective, treatment may need to be instituted soon after exposure. PMID:7383945

World War I psychoneuroses: hysteria goes to war.

PubMed

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'.

Clinical picture, epidemiology and outcome of Loa-associated serious adverse events related to mass ivermectin treatment of onchocerciasis in Cameroon

PubMed Central

Boussinesq, Michel; Gardon, Jacques; Gardon-Wendel, Nathalie; Chippaux, Jean-Philippe

2003-01-01

In August 2002, 65 cases of Loa-associated neurological Serious Adverse Events were reported after ivermectin treatment. The first signs, occurring within the 12–24 hours following treatment, included fatigue, generalized arthralgia, and sometimes agitation, mutism, and incontinence. Disorders of consciousness, including coma, generally appeared between 24 and 72 hours, and showed a rapid variation with time. The most frequent objective neurological signs were extrapyramidal. The patients presented with haemorrhages of the conjunctiva and of the retina. Biological examinations showed a massive Loa microfilaruria, the passage of Loa microfilariae into the cerebrospinal fluid, haematuria, and an increase in the C-reactive protein, all of which have been correlated with the high intensity of the initial Loa microfilaraemia. Eosinophil counts decreased dramatically within the first 24 hours, and then rose again rapidly. Electroencephalograms suggested the existence of a diffuse pathological process within the first weeks; the abnormalities disappearing after 3–6 months. Death may occur when patients are not properly managed, i.e. in the absence of good nursing. However, some patients who recovered showed sequelae such as aphasia, episodic amnesia, or extrapyramidal signs. The main risk factor for these encephalopathies is the intensity of the initial Loa microfilaraemia. The disorders of consciousness may occur when there are >50,000 Loa microfilariae per ml. The possible roles of co-factors, such as Loa strains, genetic predisposition of individuals, co-infestations with other parasites, or alcohol consumption, seem to be minor but they should be considered. The mechanisms of the post-ivermectin Loa-related encephalopathies should be investigated to improve the management of patients developing the condition. PMID:14975061

The associations of two SNPs in miRNA-146a and one SNP in ZBTB38-RASA2 with the disease susceptibility and the clinical features of the Chinese patients of sCJD and FFI.

PubMed

Gao, Chen; Shi, Qiang; Wei, Jing; Zhou, Wei; Xiao, Kang; Wang, Jing; Shi, Qi; Dong, Xiao-Ping

2018-01-02

Prion diseases are a group of fatal neurodegenerative disorders that affect humans and animals. Besides of the pathological agent, prion, there are some elements that can influence or determine susceptibility to prion infection and the clinical phenotype of the diseases, e.g., the polymorphism in PRNP gene. Another polymorphism in ZBTB38-RASA2 has been observed to be associated with the susceptibility of sporadic Creutzfeldt-Jacob disease (sCJD) in UK. MicroRNAs are endogenous small noncoding RNAs that control gene expression by targeting mRNAs and triggering either translation repression or RNA degradation. In this study, two polymorphic loci in miR-146a (rs2910164 and rs57095329) and one locus in ZBTB38-RASA2 (rs295301) of 561 Chinese patients of sCJD and 31 cases of fatal familial insomnia (FFI) were screened by PCR and sequencing. Our data did not figure out any association of those three SNPs with the susceptibility of sCJD. However, a significant association of the SNP of rs57095329 in miR-146a showed the association with the susceptibility of FFI. Additionally, the SNP of rs57095329 showed statistical significances with the appearances of mutism and the positive of cerebrospinal fluid (CSF) protein 14-3-3 in sCJD patients, while the SNP of ZBTB38-RASA2 was significantly related with the appearance of myoclonus in sCJD patients. It indicates that the SNPs of ZBTB38-RASA2 and miR-146a are not associated with the susceptibility of the Chinese sCJD patients, but may influence the appearances of clinical manifestations somehow.

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

PubMed

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 potential solutions, including modular treatment formats and innovative applications of technology.

Treatment developments and the unfolding of the quality of life discussion in childhood medulloblastoma: a review.

PubMed

Gudrunardottir, Thora; Lannering, Birgitta; Remke, Marc; Taylor, Michael D; Wells, Elizabeth M; Keating, Robert F; Packer, Roger J

2014-06-01

To describe how the quality of life (QOL) discussion in childhood medulloblastoma (MB) relates to treatment developments, survival and sequelae from 1920 to 2014. Articles containing "childhood medulloblastoma" and "quality of life" were identified in PubMed. Those containing phrases pertaining to psychological, emotional, behavioral or social adjustment in the title, abstract or keywords were selected. Inclusion of relevant older publications was assured by cross-checking references. 1920-1930s: suction, electro-surgery, kilovolt (KV) irradiation. Survival = months. Focus on operative mortality, symptoms and survival. 1940s: radiotherapy improved. 1950s: chemotherapy and intubation. Survival = years. Opinions oscillated between optimism/awareness of physical sequelae of radiotherapy. 1960s: magnified vision, ventriculo-peritoneal (VP) shunts, megavolt (MV) irradiation. Long-term survival shifted the attention towards neurological problems, disability and carcinogenesis of radiotherapy. 1970s: CT, microscope, bipolar coagulation, shunt filters, neuroanesthesia, chemotherapy trials and staging studies. Operative mortality decreased and many patients (re)entered school; emphasis on neuropsychological sequelae, IQ and academic performance. 1980s: magnetic resonance imaging (MRI), Cavitron ultrasonic aspiration (CUSA), laser surgery, hyper-fractionated radiotherapy (HFRT). Cerebellar mutism, psychological and social issues. 1990s: pediatric neurosurgery, proton beams, stem cell rescue. Reflections on QOL as such. 21st century: molecular genetics. Premature aging, patterns of decline, risk- and resilience factors. QOL is a critical outcome measure. Focus depends on survival and sequelae, determined after years of follow-up. Detailed measurements are limited by time, money and human resources, and self-reporting questionnaires represent a crude measure limited by subjectivity. Therapeutic improvements raise the question of QOL versus cure. QOL is a potential primary research endpoint; multicenter international studies are needed, as are web-based tools that work across cultures.

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

PubMed

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.

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

PubMed

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.

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

PubMed

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.

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

PubMed

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-term neurological sequelae.

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

PubMed

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.

Schizophrenia with prominent catatonic features ('catatonic schizophrenia') III. Latent class analysis of the catatonic syndrome.

PubMed

Ungvari, Gabor S; Goggins, William; Leung, Siu-Kau; Lee, Edwin; Gerevich, Jozsef

2009-02-01

No reports have yet been published on catatonia using latent class analysis (LCA). This study applied LCA to a large, diagnostically homogenous sample of patients with chronic schizophrenia who also presented with catatonic symptoms. A random sample of 225 Chinese inpatients with DSM-IV schizophrenia was selected from the long-stay wards of a psychiatric hospital. Their psychopathology, extrapyramidal motor status and level of functioning were evaluated with standardized rating scales. Catatonia was rated using a modified version of the Bush-Francis Catatonia Rating Scale. LCA was then applied to the 178 patients who presented with at least one catatonic sign. In LCA a four-class solution was found to fit best the statistical model. Classes 1, 2, 3 and 4 constituted 18%, 39.4%, 20.1% and 22.5% of the whole catatonic sample, respectively. Class 1 included patients with symptoms of 'automatic' phenomena (automatic obedience, Mitgehen, waxy flexibility). Class 2 comprised patients with 'repetitive/echo' phenomena (perseveration, stereotypy, verbigeration, mannerisms and grimacing). Class 3 contained patients with symptoms of 'withdrawal' (immobility, mutism, posturing, staring and withdrawal). Class 4 consisted of 'agitated/resistive' patients, who displayed symptoms of excitement, impulsivity, negativism and combativeness. The symptom composition of these 4 classes was nearly identical with that of the four factors identified by factor analysis in the same cohort of subjects in an earlier study. In multivariate regression analysis, the 'withdrawn' class was associated with higher scores on the Scale of Assessment of Negative Symptoms and lower and higher scores for negative and positive items respectively on the Nurses' Observation Scale for Inpatient Evaluation's (NOSIE). The 'automatic' class was associated with lower values on the Simpson-Angus Extrapyramidal Side Effects Scale, and the 'repetitive/echo' class with higher scores on the NOSIE positive items. These results provide preliminary support for the notion that chronic schizophrenia patients with catatonic features can be classified into 4 distinct syndromal groups on the basis of their motor symptoms. Identifying distinct catatonic syndromes would help to find their biological substrates and to develop specific therapeutic measures.

Transcallosal approach to tumors of the third ventricle. Surgical results and neuropsychological evaluation.

PubMed

Villani, R; Papagno, C; Tomei, G; Grimoldi, N; Spagnoli, D; Bello, L

1997-03-01

A series of 34 patients with tumours of the third ventricle were operated on by a transcallosal route. Basal extrinsic lesions compressing or invading the ventricle as well as tumours located in the pineal area were excluded from this review. Tumours were approached by a transforaminal entry in 16 cases (47%), by an interforniceal route in 11 (32%), by a subchoroidal entry in 4 (14%) and by a combined transforaminal and subchoroidal entry in 3 (9%). Four out of 34 patients were submitted to a second operation, through the same approach corridor: 2 for an incomplete removal of an intrinsic tumour and 2 for a late regrowth. Postoperative mortality rate accounted for 5.8% (2 patients). Major post operative complications were hemiparesis (4 patients) and diabetes insipidus (4 patients), that were transient in 3. Akinetic mutism like status was observed in only 1 patient. Postoperative psychic disturbances were noticed in 5 cases. Nine out of 21 patients (62%) with preoperative hydrocephalus required a permanent CSF shunt. Histology revealed that 21 tumours (62%) were intraaxial (4 pilocitic astrocytoma, 10 low grade glioma, 1 giant cell astrocytoma, 1 subependymoma, 4 ependymoma/ependymoblastoma, 1 neurocitoma) and 13 (38%) were extraaxial (8 colloid cyst, 2 craniopharingioma, 1 ectopic pituitary adenoma, 1 lymphocytic hypophysitis and 1 metastasis). Total excision of third ventricle tumours was achieved in all patients with extraaxial tumours and in 62% and 71% of intraaxial tumours with the first and second surgical procedure respectively. Ten out of 34 patients of this series were submitted to a complete neuropsychological evaluation at an interval of 2-9 years after surgery. Memory tests were pathological in 2. Disconnection signs were constantly absent. Control function were preserved. Transcallosal approach remains the best microsurgical method of third ventricle tumours treatment. This route provides the capability for a superior visualization of the entire cavity of the third ventricle through different corridors. Permanent neurological and neuropsychological deficits are not frequent. Epilepsy, that accounted for 28% in patients submitted to transcortical transventricular approach to third ventricle tumours, was never noticed in this series operated on through a transcallosal route.

The evolution of primary progressive apraxia of speech

PubMed Central

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.

2014-01-01

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 18F-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, compared to controls. Increased rates of brain atrophy over time were observed throughout the premotor cortex, as well as prefrontal cortex, motor cortex, basal ganglia and midbrain, while white matter tract degeneration spread into the splenium of the corpus callosum and motor cortex white matter. Hypometabolism progressed over time in almost all subjects. These findings demonstrate that some subjects with primary progressive apraxia of speech will rapidly evolve and develop a devastating progressive supranuclear palsy-like syndrome ∼ 5 years after onset, perhaps related to progressive involvement of neocortex, basal ganglia and midbrain. These findings help improve our understanding of primary progressive apraxia of speech and provide some important prognostic guidelines. PMID:25113789

The evolution of primary progressive apraxia of speech.

PubMed

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, compared to controls. Increased rates of brain atrophy over time were observed throughout the premotor cortex, as well as prefrontal cortex, motor cortex, basal ganglia and midbrain, while white matter tract degeneration spread into the splenium of the corpus callosum and motor cortex white matter. Hypometabolism progressed over time in almost all subjects. These findings demonstrate that some subjects with primary progressive apraxia of speech will rapidly evolve and develop a devastating progressive supranuclear palsy-like syndrome ∼ 5 years after onset, perhaps related to progressive involvement of neocortex, basal ganglia and midbrain. These findings help improve our understanding of primary progressive apraxia of speech and provide some important prognostic guidelines. © The Author (2014). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

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

PubMed

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 out-of-court settlement was 35% and the time limit for settlement by the justice was on average 6 months with extremes of one month and 24 months. The average cost of care was estimated at 17,010 CFA francs (26 euros) taking into account the consumables used for clinical examination (sterile gloves, catheter, syringe), analysis and prescription drugs. The sexual abuse of minors is a disturbing reality that raises rightly universal reprobation. In Senegal, this mainly affects children and its magnitude is increasing over the years. Improved support for victims necessarily involves raising public awareness through the media and the development of specialized structures in the management of sexual abuse. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

[Functional pathophysiology of consciousness].

PubMed

Jellinger, Kurt A

2009-01-01

Consciousness (Latin conscientia "moral conscience"), according to the English philosopher John Locke (1632-1704) [103], is the awareness of all that occurs in the mind of a person, whereas the American philosopher John Searle (2000) defined it as "inner qualitative, subjective states and processes of awareness". In modern science it is defined as a continuous state of full awareness of the Self and one's relationship to the external and internal environment, describing the degree of wakefulness in which an organism recognizes stimuli. This widely discussed biological term for complex neuronal processes that allow an individuum to recognize itself and its environment and to act accordingly, has been and still is the subject of much research in philosophy and natural/neuroscience. Its definition is often used for awareness and recognition, too. While the Egyptians in the papyrus Edwin Smith already recognized the brain as the seat of consciousness, René Descartes (1644 [36]) believed its special structure should be "a small gland in the middle", but the anatomical structures and physiological processes involved in consciousness were elucidated only in the middle of the 20th century. Neuronal substrates include several functional networks that are hierarchically organized and cooperate functionally. The lowest level is the mesencephalic formatio reticularis and its projections to the thalamus that were identified als ascending reticular system (ARAS) by the classical experiments of Moruzzi and Magoun, whereas later analyses of patients with impaired consciousness provided further insights. The mesencephalic ARAS as motor of the function of higher structures projects 1. via the reticular thalamus diffusely to the cortex, 2. via hypothalamus to the basal forebrain and limbic system, and 3. to the medial raphe of the brainstem and locus coeruleus and their diffuse cortical projections. The reticular system is stimulated directly and indirectly via numerous collaterals 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.

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

PubMed

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 adding an affective dimension which is considered specific. Clinical forms are differentiated according to evolution: acute, chronic and periodic forms exist; and symptomatology: excited catatonias have a best prognostic than retarded catatonias. Malignant catatonia is the most studied form because of its severity and high rate of mortality (25%); catatonic patients develop autonomic disturbances with labile blood pressure, hyperthermia, diaphoresis, etc. Malignant catatonia requires ECT intervention in emergency. While catatonias due to general medical conditions are well admitted (first concerned are neurologic etiologies) and concern 14,1% of catatonias, psychiatric comorbidity remains a problem. The documented decline in the proportion of patients with schizophrenia diagnosed as catatonic is congruent with the fact that most studies highlight the strong association between catatonia and mood disorders. However, customary clinical practice continues to over value diagnostic of schizophrenia because catatonic symptoms are recognized as schizophrenic and schizophrenia corresponds to a pharmacological target. Other authors stress that on average 20 to 40% of catatonias are idiopathic. Conceptual proximity between catatonic symptomatology and extrapyramidal syndrome could give some ways for neurobiological grasp of the trouble; mesolimbic and mesostriatal dopaminergic imbalance in a frontal lobe-basal ganglia-brainstem system is supposed to be involved. Treatment procedure could be standardized as follows: 1) Withhold neuroleptic medication. Those drugs are proven to be lethal when catatonic symptoms are developed; 2) Investigations to exclude treatable physical disorders (including standard blood laboratory tests, urinary drug screening, electroencephalogram and brain computerized tomography); 3) Trial of lorazepam. This therapeutic is safe and 80% effective. We propose to administer an initial oral 2,5 mg challenge; catatonic signs are rated after the first hours. If necessary, the patient could receive 3 mg/day with a 6-day full dose treatment and then, treatment would progressively be reduced; 4) If the patient failed to respond to lorazepam, ECT are needed; 5) Earlier use of ECT is recommended if autonomic instability or hyperthermia appears and malignant catatonia is suspected. In conclusion, catatonia has always had an unstable and blurred place in the psychiatric nosography since its first description. It has been incorporated within the group of schizophrenias and underdiagnosed for a long time, but has been predominantly associated with mood disorders for the last ten years. Psychopathological considerations, particularly on cognitive and affective status of catatonic patients, should clarify the nosologic discussion.

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

PubMed

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

2003-01-01

Many authors have described these last years the difficulty to establish a differential diagnosis between schizophrenia and frontotemporal dementia. However treatment and prognosis of these two separate diseases are not the same. Schizophrenia is a chronic syndrome with an early onset during teenage or young adulthood period and the major features consist of delirious ideas, hallucinations and psychic dissociation. However a large variety of different symptoms describes the disease and creates a heterogeneous entity. The diagnosis, exclusively defined by clinical signs, is then difficult and has led to the research of specific symptoms. These involve multiple psychological processes, such as perception (hallucinations), reality testing (delusions), thought processes (loose associations), feeling (flatness, inappropriate affect), behaviour (catatonia, disorganization), attention, concentration, motivation (avolition), and judgement. The characteristic symptoms of schizophrenia have often been conceptualised as falling into three broad categories including positive (hallucination, delision), negative (affective flattening, alogia, avolition) and disorganised (poor attention, disorganised speech and behaviour) symptoms. No single symptom is pathogonomonic of schizophrenia. These psychological and behavioural characteristics are associated with a variety of impairments in occupational or social functioning. Cognition impairments are also associated with schizophrenia. Since the original clinical description by Kraepelin and Bleuler, abnormalities in attentional, associative and volitional cognitive processes have been considered central features of schizophrenia. Long term memory deficits, attentional and executive dysfunctions are described in the neurocognitive profile of schizophrenic patients, with a large degree of severity. The pathophysiology of schizophrenia is not well known but may be better understood by neuronal dysfunctions rather than by a specific 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 old non-married man is admitted in mental health district of a general hospital for behavioural disturbances that include repeated aggressions towards his family. At initial interview visual and auditives hallucinations are described. The patient doesn't care about these abnormalities and a poverty of speech is observed. The affects, globally blunted, show some degree of sadness however. The patient's birth and early development were unremarkable. At the age of 26, the patient dismissed from his job because of poor performance and absenteeism. He spent a lot of time watching TV, showed poverty of speech and become sometimes angry and violent without an explanation. He was hospitalised for several months and a schizophrenia including predominant negative features, hallucinations and delusion was diagnosed. He was treated with bromperidol, could go back to home and was followed by a general practitioner for 8 years. The patient had a stereotyped way of life during these years with a poor communication and little activity. During the months preceding the current hospitalisation, these characteristics and avolition emphasised, urinary incontinence appeared. The patient receives risperidone 8 mg/day associated with citalopram 40 mg/day during several months of hospitalisation. No significant evolution is observed regarding apathic and stereotyped way of live. The capacity of communication remains very poor. Neurocognitive assessments reveal multiple and severe dysfunctions. Memory, executive and attentional tasks are extremely disturbed. Physical and neurological examinations reveal an isolated bilateral Babinski sign. Cerebral scanner and magnetic resonance show bifrontal atrophy and PET scan is normal. There are no significant abnormalities found on blood and urine samples and on lumbar puncture. The patient is sent to a chronic neuropsychiatric hospital and the treatment is stopped. One year later, a comparative evaluation is realised. The general clinical state shows no evolution. Neurocognitive assessments are repeated and severe dysfunctions are observed with more perseverations. A diagnosis of FTLD for this patient can be discussed regarding clinical features, neurocognitive testings and neuroradiological findings. Schizophrenia is a major differential diagnosis. Psychotic symptoms like hallucinations and age of onset are essential observations for the diagnosis of schizophrenia but can not exclude FTLD. Memory, intellectual functions, executive and attentional abilities may all be disturbed in schizophrenia and FTLD. Cerebral abnormalities well established in schizophrenia are lateral ventricles enlargements. Frontal lobar atrophy is a major argument for FTLD and is only a sporadic finding in schizophrenic populations. Schizophrenia and FTLD could be comorbid diseases by several ways. A differential diagnosis between schizophrenia and FTLD is difficult to establish. Schizophrenia is a heterogeneous disease with a large variety of cognitive dysfunctions. Neurocognitive tools may improve our knowledge of schizophrenia.

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

PubMed

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

2012-05-01

Maximum contaminant levels are used to control potential health hazards posed by chemicals in drinking water, but no primary national or international limits for aluminum (Al) have been adopted. Given the differences in toxicological profiles, the present evaluation derives total allowable concentrations for certain water-soluble inorganic Al compounds (including chloride, hydroxide, oxide, phosphate and sulfate) and for the hydrated Al silicates (including attapulgite, bentonite/montmorillonite, illite, kaolinite) in drinking water. The chemistry, toxicology and clinical experience with Al materials are extensive and depend upon the particular physical and chemical form. In general, the water solubility of the monomeric Al materials depends on pH and their water solubility and gastrointestinal bioavailability are much greater than that of the hydrated Al silicates. Other than Al-containing antacids and buffered aspirin, food is the primary source of Al exposure for most healthy people. Systemic uptake of Al after ingestion of the monomeric salts is somewhat greater from drinking water (0.28%) than from food (0.1%). Once absorbed, Al accumulates in bone, brain, liver and kidney, with bone as the major site for Al deposition in humans. Oral Al hydroxide is used routinely to bind phosphate salts in the gut to control hyperphosphatemia in people with compromised renal function. Signs of chronic Al toxicity in the musculoskeletal system include a vitamin D-resistant osteomalacia (deranged membranous bone formation characterized by accumulation of the osteoid matrix and reduced mineralization, reduced numbers of osteoblasts and osteoclasts, decreased lamellar and osteoid bands with elevated Al concentrations) presenting as bone pain and proximal myopathy. Aluminum-induced bone disease can progress to stress fractures of the ribs, femur, vertebrae, humerus and metatarsals. Serum Al ≥100 µg/L has a 75-88% positive predictive value for Al bone disease. Chronic Al 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 mg/day in children of 3-6 years, 4,200 mg/day in children of 6-12 years, and 9,000 mg/day in adults. Chronic ingestion of insoluble hydrated Al silicates (in kg) can result in disturbances in iron and potassium status, primarily as a result of clay binding to intestinal contents and enhanced fecal iron and zinc elimination. Sufficiently high doses of ingested Al silicates (≥50 g/day) over prolonged periods of time can elicit a deficiency anemia that can be corrected with oral Fe supplements. There is essentially no systemic Al uptake after ingestion of the hydrated Al silicates. Rats fed up to 20,000 ppm Ca montmorillonite (equivalent to 1,860 ppm total Al as the hydrated Al silicate) for 28 weeks failed to develop any adverse signs. The results of dietary Phase I and II clinical trials conducted in healthy adult volunteers over 14 days and 90 days with montmorillonite found no adverse effects after feeding up to 40 mg/kg-day as Al. Since the Al associated with ingestion of hydrated Al silicates is not absorbed into the systemic circulation, the hydrated Al silicates seldom cause medical problems unless the daily doses consumed are substantially greater than those used clinically or as dietary supplements. A no-observable-adverse-effect-level (NOAEL) of 13 mg/kg-day as total Al can be identified based on histologic osteomalacia seen in adult hemodialysis patients given Al hydroxide for up to 7 years as a phosphate binder. Following U.S. EPA methods for calculation of an oral reference dose (RfD), an intraspecies uncertainty factor of 10x was applied to that value results in a chronic oral reference dose (RfD) of 1.3 mg Al/kg-day; assuming a 70-kg adult consumes 2 L of drinking water per day and adjusting for a default 20% relative source contribution that value corresponds to a drinking water maximum concentration of 9 mg/L measured as total Al. A chronic NOAEL for montmorillonite as representative of the hydrated Al silicates was identified from the highest dietary concentration (20,000 ppm) fed in a 28-week bioassay with male and female Sprague-Dawley rats. Since young rats consume standard laboratory chow at ~23 g/day, this concentration corresponds to 56 mg Al/kg-day. Application of 3x interspecies uncertainty factor and a 3x factor to account for study duration results in a chronic oral RfD of 6 mg Al/kg-day. Of note, this RfD is 5-10 fold less than oral doses of Al silicates consumed by people who practice clay geophagy and it corresponds to a maximum drinking water concentration of 40 mg Al/L. To utilize the values derived here, the risk manager must recognize the particular product (e.g., alum) or source (e.g., groundwater, river water, clay or cement pipe) of the Al found in tap water, apply the appropriate analytical methods (atomic absorption, energy dispersive X-ray diffraction, infrared spectral analysis and/or scanning transmission electron microscopy) and compare the results to the most relevant standard. The drinking water concentrations derived here are greater than the U.S. EPA secondary maximum contaminant level (MCL) for total Al of 0.05-0.2 mg/L [40 CFR 143.3]. As such, domestic use of water with these concentrations is likely self-limiting given that its cloudy appearance will be greater than the maximum permitted (0.5-5.0 nephalometric turbidity units; 40 CFR Parts 141 and 142). Therefore, the organoleptic properties of Al materials in water determine public acceptance of potable water as contrast to any potential health hazard at the concentrations ordinarily present in municipal drinking water.