Stefanie Keulen

Developmental Foreign Accent Syndrome: Report of a New Case

This paper presents the case of a 17-year-old right-handed Belgian boy with developmental FAS and comorbid developmental apraxia of speech (DAS). Extensive neuropsychological and neurolinguistic investigations demonstrated a normal IQ but impaired planning (visuo-constructional dyspraxia). A Tc-99m-ECD SPECT revealed a significant hypoperfusion in the prefrontal and medial frontal regions, as well as in the lateral temporal regions. Hypoperfusion in the right cerebellum almost reached significance. It is hypothesized that these clinical findings support the view that FAS and DAS are related phenomena following impairment of the cerebro-cerebellar network.

Consensus Paper: Cerebellum and Emotion

Over the past three decades, insights into the role of the cerebellum in emotional processing have substantially increased. Indeed, methodological refinements in cerebellar lesion studies and major technological advancements in the field of neuroscience are in particular responsible to an exponential growth of knowledge on the topic. It is timely to review the available data and to critically evaluate the current status of the role of the cerebellum in emotion and related domains. The main aim of this article is to present an overview of current facts and ongoing debates relating to clinical, neuroimaging, and neurophysiological findings on the role of the cerebellum in key aspects of emotion. Experts in the field of cerebellar research discuss the range of cerebellar contributions to emotion in nine topics. Topics include the role of the cerebellum in perception and recognition, forwarding and encoding of emotional information, and the experience and regulation of emotional states in relation to motor, cognitive, and social behaviors. In addition, perspectives including cerebellar involvement in emotional learning, pain, emotional aspects of speech, and neuropsychiatric aspects of the cerebellum in mood disorders are briefly discussed. Results of this consensus paper illustrate how theory and empirical research have converged to produce a composite picture of brain topography, physiology, and function that establishes the role of the cerebellum in many aspects of emotional processing.

Psychogenic Foreign Accent Syndrome: A New Case

This paper presents the case of a 33-year-old, right-handed, French-speaking Belgian lady who was involved in a car accident as a pedestrian. Six months after the incident she developed a German/Flemish-like accent. The patients medical history, the onset of the FAS and the possible psychological causes of the accent change are analyzed. Relevant neuropsychological, neurolinguistic, and psychodiagnostic test results are presented and discussed. The psychodiagnostic interview and testing will receive special attention, because these have been underreported in previous FAS case reports. Furthermore, an accent rating experiment was carried out in order to assess the foreign quality of the patients speech. Pre- and post-morbid spontaneous speech samples were analyzed phonetically to identify the pronunciation characteristics associated with this type of FAS. Several findings were considered essential in the diagnosis of psychogenic FAS: the psychological assessments as well as the clinical interview confirmed the presence of psychological problems, while neurological damage was excluded by means of repeated neuroimaging and neurological examinations. The type and nature of the speech symptoms and the accent fluctuations associated with the patients psychological state cannot be explained by a neurological disorder. Moreover, the indifference of the patient toward her condition may also suggest a psychogenic etiology, as the opposite is usually observed in neurogenic FAS patients.

The Posterior Fossa and Foreign Accent Syndrome : Report of Two New Cases and Review of the Literature

Foreign accent syndrome is a rare motor speech disorder that causes patients to speak their language with a non-native accent. In the neurogenic condition, the disorder develops after lesions in the language dominant hemisphere, often affecting Broca’s area, the insula, the supplementary motor area and the primary motor cortex. Here, we present two new cases of FAS after posterior fossa lesions. The first case is a 44-year-old, right-handed, Dutch-speaking man who suffered motor speech disturbances and a left hemiplegia after a pontine infarction. Quantified SPECT showed a bilateral hypoperfusion in the inferior lateral prefrontal and medial inferior frontal regions as well as a significant left cerebellar hypoperfusion. Further clinical investigations led to an additional diagnosis of brainstem cognitive affective syndrome which closely relates to Schmahmann’s syndrome. The second patient was a 72-year-old right-handed polyglot English man who suffered a stroke in the vascular territory of the left posterior inferior cerebellar artery (PICA) and developed a foreign accent in his mother tongue (English) and in a later learnt language (Dutch). In this paper, we discuss how the occurrence of this peculiar motor speech disorder can be related to a lesion affecting the posterior fossa structures.

Cerebellar induced differential polyglot aphasia: A neurolinguistic and fMRI study

HighlightsThe bilingual language network also involves domain‐general executive control areas.The cerebellum might contribute to multilingual language control via the cerebello‐cerebral network.Left cerebellar damage in right‐handed subjects can elicit differential polyglot aphasia. Abstract Research has shown that linguistic functions in the bilingual brain are subserved by similar neural circuits as in monolinguals, but with extra‐activity associated with cognitive and attentional control. Although a role for the right cerebellum in multilingual language processing has recently been acknowledged, a potential role of the left cerebellum remains largely unexplored. This paper reports the clinical and fMRI findings in a strongly right‐handed (late) multilingual patient who developed differential polyglot aphasia, ataxic dysarthria and a selective decrease in executive function due to an ischemic stroke in the left cerebellum. fMRI revealed that lexical‐semantic retrieval in the unaffected L1 was predominantly associated with activations in the left cortical areas (left prefrontal area and left postcentral gyrus), while naming in two affected non‐native languages recruited a significantly larger bilateral functional network, including the cerebellum. It is hypothesized that the left cerebellar insult resulted in decreased right prefrontal hemisphere functioning due to a loss of cerebellar impulses through the cerebello‐cerebral pathways.

Foreign Accent Syndrome As a Psychogenic Disorder: A Review

In the majority of cases published between 1907 and 2014, FAS is due to a neurogenic etiology. Only a few reports about FAS with an assumed psychogenic origin have been published. The present article discusses the findings of a careful database search on psychogenic FAS. This review may be particularly relevant as it is the first to analyze the salient features of psychogenic FAS cases to date. This article hopes to pave the way for the view that psychogenic FAS is a cognate of neurogenic FAS. It is felt that this variant of FAS may have been underreported, as most of the psychogenic cases have been published after the turn of the century. This review may improve the diagnosis of the syndrome in clinical practice and highlights the importance of recognizing psychogenic FAS as an independent taxonomic entity.

Alexia: Diagnosis, treatment and theory by Leff, A., & Starrfelt, R.

In Alexia: Diagnosis, Treatment and Theory, Alexander Leff (University College London) and Randi Starrfelt (University of Copenhagen) aim to uncover the intricate theoretic basis underlying alexia: “deficits in reading following damage to the brain in previously literate individuals” (p. 2). The book focuses on the acquired alexias, as opposed to the developmental dyslexias, which disturb the development of reading skills during childhood. In How Do We Read? (Chapter 1), the authors give a concise but complete overview of the basic principles necessary to understand the history and the conceptualisation of the term alexia. They dedicate ample attention to the different methodological angles from which these reading disorders can be examined and provide the reader with very comprehensive insights into the baffling process of reading itself, including a summary of the more recent (cognitive) models of reading (pp. 7–14). This account very much aids the comprehension of the experimental designs and case studies reported in the following chapters. As befits a good scientific approach, Leff and Starrfelt clearly explain their own methodological approach, stating that in their analyses the neuropsychological distinction between central and peripheral alexias is used, instead of the neurological distinction between alexia with and without agraphia (also known as pure alexia). The authors provide the readers with a succinct state of the art on reading and reading processes, including a digression on findings in neuroimaging studies (pp. 16–21) and a short introduction to the neuroanatomy of eye movements (pp. 23–25). In Chapters 2–4, the different types of alexias are presented in great detail. In a very consistent and clear way, Leff and Starrfelt opt for a stringent deductive analysis of each type of alexia, starting off with a clear definition, followed by an overview of the (possible) pathological substrate(s) and lesion locations, the repercussions of the disorders on reading and reading processes, the possible theoretical explanations as to where and how the reading problems emerge, and, lastly, an evaluation of the possible therapeutic remediation strategies. This provides a methodologically creditable approach that renders their work very user-friendly. The first type of alexia dealt with is hemianopic alexia (Chapter 2). Due to this visuoperceptive impairment, only a part of a word or a sentence is perceived and read. It seems quite obvious that this is especially inconvenient for patients with a right-sided hemianopia, since—at least in Western culture—it is common to read from left to right. Leff and Starrfelt extensively describe how visual field impairments—and by inference, sparing—can be measured. They give an outline of the Humphrey Automated Field Test, as an example of a clinical test for macular sparing, and provide ample figures as a means of illustration and concretisation of the more abstract theoretic rendering (pp.