Partial Seizure with Aphasic Speech Arrest Caused by Watching a Popular Animated TV Program

On the evening of December 16, 1997, about 700 children across Japan were hospitalized because of convulsive seizures or vomiting experienced while watching a popular animated TV program that included blue and red stimuli that alternated at 12 flashes per second. In one case, an 11-year-old girl developed a hallucination in the right visual field and a subsequent cramp on the right side of her face, with aphasic speech arrest. She had no history of seizures. Her electroencephalogram (EEG) showed normal background activity and no epileptiform discharges. Intermittent photic stimulation provoked a photoparoxysmal response. Her main clinical manifestation was a TV-induced left occipital lobe seizure spreading toward the left inferior frontal lobe. This suggested a functional link from the occipital lobe to the frontal operculum.     

Acquired Aphasia with Epilepsy–Landau-Kleffner Syndrome

Landau-Kleffner syndrome is characterized by long-lasting acquired aphasia associated with seizures and EEG abnormalities. Three new cases of this rare syndrome are reported from India.     

Reciprocal scaffolding treatment: A person with aphasia as clinical teacher

Background: Reciprocal Scaffolding Treatment (RST) is one of several potentially beneficial life participation approaches for aphasia. In RST, treatment occurs during genuine, relevant, and context dependent interactions that represent goals at the activity and participation levels of the World Health Organization International Classification of Functioning, Disability and Health (ICF; World Health Organization, 2001) and is based on an apprenticeship model of learning where novices are taught skills by a more skilled partner. RST was used to construct a communicatively challenging environment in which an expert with aphasia (AE) taught novices (graduate student clinicians) how to communicate with persons with aphasia in the context of conversation group treatment sessions. This is in contrast to many treatment techniques when the person with aphasia is the novice who is trying to relearn communication skills during treatment sessions with a speech‐language pathologist as the expert.

Aims: The goal of the study was to investigate the effect of RST on improvement in word retrieval and conversational components in an individual with anomic aphasia.

Methods & Procedures: This was a case study using pretreatment – post treatment assessment. The independent variable was application of RST and the contextual variables were the presence of novices (graduate student clinicians) and unfamiliar conversation partners (undergraduate speech‐language pathology students). The dependent variables were scores on a word fluency task (FAS) and conversational measures (CIUs and TTR). Over the course of a seven week training period, AE taught communication strategies to four novice graduate student clinicians, who used the strategies in conversation groups composed of 3 to 4 persons with aphasia.

Outcomes & Results: The individual with aphasia made positive changes in word fluency, Correct Information Units and Type‐Token Ratio.

Conclusions: These findings, while preliminary in nature, show how the authentic use of language in structured reciprocal interactions such as teaching may improve language. A reciprocal teaching environment carries with it the expectation that at least one participant have an intent to participate as an expert in order to convey information to novices. We speculate that the combination of reciprocal interaction and the intent to convey information, in this case in a unique manner, support improved language skills.     

Behavioural aspects of Pollitt syndrome: a 32-year follow-up of a case described by R. J. Pollitt and colleagues in 1968

In 1968, R. J. Pollitt and colleagues described a syndrome characterized by abnormally brittle, sulphur‐deficient hair (trichothiodystrophy), intellectual disability (ID) and growth retardation. One of the two siblings originally described by the above authors has recently been re‐assessed by the present authors following a referral for advice about ritualistic behaviours. Her current clinical features are described, and the literature concerning trichothiodystrophy and ID is summarized.     

Levodopa-induced dyskinesia: clinical observations

Summary In 144 patients receiving prolonged treatment with levodopa for Parkinson's disease, an attempt was made to establish possible correlations between the incidence of levodopa-induced dyskinesias and the age of the patient at the onset of the disease, the clinical form of the disease, the duration of symptoms before initiation of the levodopa therapy, the duration of the levodopa therapy and the influence of the concomitant treatment. Levodopa-induced dyskinesia was observed in 92 patients (64%). The age at onset of the disease of patients with dyskinesia was significantly different from the age at onset of those without dyskinesia, the means being 54.8 and 68.9 years respectively. Levodopa-induced dyskinesia occurred less often in the group with preponderant tremor than in those with preponderant bradykinesia (29% vs. 69%). The patients treated with levodopa from the very beginning of their disease were less susceptible to dyskinesia than those who had parkinsonism for some time before receiving levodopa. The influence of the duration of levodopa treatment on the manifestation of dyskinesia could not be confirmed because this side-effect usually appeared during the first year of treatment. The concomitant anti-parkinsonian treatment appeared to have no influence on the incidence of dyskinesia. Biochemical and practical implications of these observations are discussed.     

A case of unilateral opercular syndrome associated with a subcortical lesion

Summary A patient who developed a unilateral opercular syndrome following a cerebrovascular accident is described. Computed tomography showed that the lesion did not affect the opercular cortex, but involved deep white matter and the head of the caudate nucleus of the left hemisphere. Persistent hypophonia and transient aphasia were associated. Comparison with previous cases is discussed.     

Gerstmann综合征临床与神经心理学研究

目的研究Gerstmann综合征(GS)的临床与神经心理学特点。方法采用汉语失语症成套测评(ABC)、书写及视空间检查,对259例经CT或MRI检查确诊为脑卒中或脑损伤的患者进行评价。结果 (1)259例患者中有19例患有Gerstmann综合征(GS),其中9例有GS四主征、10例有GS的核心症状、命名性失语(AA)5例、经皮质运动性失语(TCM)2例、经皮质感觉性失语(TCS)4例、Wernicke失语3例、构音障碍2例、无失语5例;(2)病变部位:左顶枕、左侧颞顶、左侧颞顶枕、左侧额顶枕和左侧基底节共17例,右侧基底节、顶叶损害2例。结论 (1)Gerstmann综合征不是一个独立的综合征;(2)其病变部位不仅仅限于优势半球顶叶。     

The role of Broca's area in regular past-tense morphology: an event-related potential study

It has been suggested that damage to anterior regions of the left hemisphere results in a dissociation in the perception and lexical activation of past-tense forms. Specifically, in a lexical-decision task in which past-tense primes immediately precede present-tense targets, such patients demonstrate significant priming for irregular verbs (spoke-speak), but, unlike control participants, fail to do so for regular verbs (looked-look). Here, this behavioral dissociation was first confirmed in a group of eleven patients with damage to the pars opercularis (BA 44) and pars triangularis (BA 45) of the left inferior frontal gyrus (i.e., Broca's area). Two conditions containing word-onset orthographic-phonological overlap (bead-bee, barge-bar) demonstrated that the disrupted regular-verb priming was accompanied by, and covaried with, disrupted ortho-phonological priming, regardless of whether prime stimuli contained the regular inflectional rhyme pattern. Further, the dissociation between impaired regular-verb and preserved irregular-verb priming was shown to be continuous rather than categorical; priming for weak-irregular verbs (spent-spend) was intermediate in size between that of regular verbs and strong verbs. Such continuous dissociations grounded in ortho-phonological relationships between present- and past-tense forms are predicted by single-system, connectionist approaches to inflectional morphology and not predicted by current dual-system, rule-based models. Event-related potential data demonstrated that N400 priming effects were intact for both regular and irregular verbs, suggesting that the absence of significant regular-verb priming in the response time data did not result from a disruption of lexical access, and may have stemmed instead from post-lexical events such as covert articulation, segmentation strategies, and/or cognitive control.     

The international prevalence, epidemiology, and clinical phenomenology of Tourette syndrome: A cross-cultural perspective

The overall international prevalence of Tourette syndrome (TS) is 1% in the majority of cultures of the world. Both TS and tics are certainly more obvious and may be more common in younger people. Moreover, TS is seen less frequently in some cultures. However, in all cultures where it has been reported, the phenomenology is similar, highlighting the biological underpinnings of the disorder. This article reviews the international prevalence, epidemiology, and clinical phenomenology of TS, from a cross-cultural perspective.     

拟诊单纯疱疹病毒脑炎所致颞叶受累的失语症二例

目的 探讨左颞叶病灶与失语表现之间的关系.方法 对2例单纯疱疹病毒脑炎所致的主要表现为失语的患者进行汉语失语检查和影像学分析.结果 以左颞上回后部受累为主者与以左颞上回前部及颞中回受累者失语类型完全不同,前者属于不能分型,而后者为典型的Wernicke失语.结论 颞上回后部受累为主所致失语者听理解障碍较轻,颞上回前部受累为主并累及颞中回可导致听理解的严重受损,左颞叶的不同部位在语言形成过程中起着不同的作用.     

Approximate quantities and exact number words: dissociable systems

Numerical abilities are thought to rest on the integration of two distinct systems, a verbal system of number words and a non-symbolic representation of approximate quantities. This view has lead to the classification of acalculias into two broad categories depending on whether the deficit affects the verbal or the quantity system. Here, we test the association of deficits predicted by this theory, and particularly the presence or absence of impairments in non-symbolic quantity processing. We describe two acalculic patients, one with a focal lesion of the left parietal lobe and Gerstmann’s syndrome and another with semantic dementia with predominantly left temporal hypometabolism. As predicted by a quantity deficit, the first patient was more impaired in subtraction than in multiplication, showed a severe slowness in approximation, and exhibited associated impairments in subitizing and numerical comparison tasks, both with Arabic digits and with arrays of dots. As predicted by a verbal deficit, the second patient was more impaired in multiplication than in subtraction, had intact approximation abilities, and showed preserved processing of non-symbolic numerosities.     

Changes in aphasic discourse after contrasting treatments for anomia

Background: Discourse analysis is a key element in determining treatment effects. However, it is extremely labour intensive, requiring in‐depth knowledge of linguistics and aphasiology; thus, it is often neglected in the analysis of treatment outcomes. A clinically practical method of discourse analysis would be beneficial for evaluation and determination of treatment efficacy.

Aims: The current study investigates changes in discourse content following contrasting treatments for anomia using grammatical analysis. In addition, we pilot the use of a new information measure.

Methods & Procedures: We compare discourse changes after a gestural + verbaltreatment and a semantic‐phonologic treatment for nouns and verbs on two groups of individuals with aphasia. Analyses compared discourse samples from 14 participants taken at baseline, post‐phase 1, and post‐phase 2. In addition to traditional measures such as number of nouns, verbs, and sentence types, a new measure of information is introduced, the Utterance with New Information (UNI). The UNI is designed to assess content in non‐propositional, impaired speech in open‐ended discourse.

Outcomes & Results: Noun production increased in participants of both treatments, whereas grammatical sentences increased only in participants of the semantic‐phonologic treatment. Production of UNIs increased in participants of both treatments as well as over time.

Conclusions: This study demonstrates that a few easily counted measures of discourse production can provide clinically useful information for the clinician. Moreover, these findings suggest discourse analysis is a viable method of determining treatment outcomes especially given that improving discourse is the ultimate goal of all aphasia treatments.     

Unblending Borderline Personality and Bipolar Disorders

Borderline Personality (BPD) and Bipolar (BP) disorders stimulate an academic debate between their distinction and the inclusion of Borderline in the Bipolar spectrum. Opponents to this inclusion attribute the important differences and possible diagnostic incomprehension to overlapping symptoms. We tested 248 Borderline and 113 Bipolar patients, consecutively admitted to the Psychiatric Unit, through DSM-IV Axis I and II Disorders (SCID-I/II), Hamilton Depression Rating Scale (HAM-D), Hamilton Anxiety Rating Scale (HAM-A), Young Mania Rating Scale (YMRS) and Borderline Personality Disorder Severity Index-IV (BPDSI-IV). All the tests statistically discriminated the disorders (p < 0.0001). Overlapping symptoms resulted significantly different (impulsivity = 5.32 in BPD vs 1.55 in BP, p < 0.0001; emotional instability = 7.11 in BPD vs 0.55 in BP, p < 0.0001) and the range of their scores gives the opportunity for an even more precise discrimination. Distinctive traits (e.g. irritability or sexual arousal) are also discussed in order to try to qualify the core of these disorders to a higher degree. Comorbidity proves to be extremely small (3.6%). However, Borderline patients with manic features offer a privileged point of view for a deeper analysis. This allows for the possibility of a more precise examination of the nature and load of each symptom. Borderline Personality and Bipolar Disorders can be distinguished with high precision using common and time-sparing tests. The importance of discriminating these clinical features may benefit from this evidence.     

Landau-Kleffner Syndrome: Consistent Response to Repeated Intravenous γ-Globulin Doses: A Case Report

Summary: Purpose: Although several treatments have been tried for Landau-Kleffner syndrome (LKS) too many patients are refractory to known therapies. We report an 8–year-old girl who failed other therapies but who had a consistent response after treatment with intravenous (i.v.) γ-globulin. Methods: We monitored the girl from the age of 6 years, when she presented with a 6–month history of loss of language with normal hearing, normal brain magnetic resonance imaging (MRI), increased cerebrospinal fluid (CSF) IgG index, and an EEG showing almost continuous, predominantly left-sided spike- and slow-wave complexes. She had no clinical seizures and did not respond to consecutive trials of valproate (VPA), clonazepam (CZP), prednisone, and carbamazepine (CBZ). She received three courses of intravenous (i.v.) γ-globulin; after each course, clinical and electrographic improvement lasted a few months. After each of the initial two eourses, clinical improvement lasted 3–4 months but was followed by recurrence of the spikes on the EEG and by speech deterioration. Results: However, her last remission has been continuous for the past 16 months. Her CSF IgG index became normal after the first i.v. γ-globulin infusion. Conclusions: Based on our experience with this patient and on other investigators' experience, we believe that further research into immunologic mechanisms and therapies of this syndrome are warranted.     

Successful use of intravenous immunoglobulin as initial monotherapy in Landau-Kleffner syndrome

PURPOSE: There is a need for new and more effective therapies for Landau-Kleffner syndrome. In this article we present the first case in which a patient with Landau-Kleffner syndrome was given intravenous immunoglobulin (IVIG) as his first and only therapy and responded to it. METHODS: This previously healthy, left-handed boy presented at 31 months of age with a 3-month history of auditory agnosia, behavioral abnormalities, and progressive, eventually complete loss of speech. Electroencephalography (EEG) showed frequent and, in sleep, continuous right central and temporal spike slow wave discharges. Metabolic workup, magnetic resonance imaging, and auditory evoked potentials were normal. Cerebrospinal fluid IgG index was high (18%). The patient was treated with IVIG, as his initial and only therapy, receiving 500 mg/kg/day over four consecutive days. RESULTS: On the third day of IVIG, the patient started using single words, and on the fourth, two-word sentences. Two weeks later his speech and behavior returned to normal. At the end of 4 days of IVIG therapy, EEG was within normal limits. Two months later, however, he had a severe relapse clinically and by EEG. He promptly responded to another course of IVIG. A subsequent cerebrospinal fluid IgG index showed normalization (6%). Three months later he had essentially normal speech and behavior. CONCLUSIONS: Repeated, immediate, and remarkable clinical and EEG responses of this patient suggest that IVIG was helpful as first-line therapy in the treatment of Landau-Kleffner syndrome. It also supports the hypothesis that immunological mechanisms contributed to his symptoms.     

非流利失语型额颞叶痴呆患者神经心理学特征及皮质代谢功能变化

目的 正规的认知功能评价结合脱氧葡萄糖正电子发射断层摄影术(FDG-PET)检查和影像学改变,分析非流利失语型额颞叶痴呆(FTD)患者的神经心理学特征.方法 选择临床、影像、皮质葡萄糖代谢功能以及神经心理学评价均符合非流利失语型FTD患者,动态评价其神经语言学以及多种认知功能.按其特殊的神经心理学特点结合FDG-PET分析神经心理学改变的皮质基础.结果 5例患者的核心症状均为隐袭起病、慢性进行性的非流利型失语,伴随的突出改变为视空间、字词级阅读保留好,记忆相对好,以非语言记忆保留更突出.生活适应能力损害相对晚.患者均有相应的皮质糖代谢改变基础.结论 非流利失语型FTD患者的视空间能力、字词级阅读能力以及生活适应能力损害晚,与相应的皮质代谢功能相关,可以帮助照料者有效指导、调整患者的适应能力训练.     

抽动秽语综合征的临床特征分析

目的探讨抽动秽语综合征(Tourette‘s syndrome，TS)的临床特征。方法对69例抽动秽语综合征患者的临床资料进行分析。结果TS占同期儿童神经精神疾病的0.16％。早期均为多发性抽动，73．9％出现发声抽动，42％出现模仿言语；37．7％脑电图异常；47．8％有脑外伤史、难产史、早产史、感染史和母孕期间出现重大生活事件等；7.2％患儿家族中有TS、癫癇和精神分裂症患者。结论本征可能是生物、心理与环境因素相互作用所致。预后良好。