The ontogenesis of language impairment in autism: a neuropsychological perspective.

Autistic Disorder (AD) is a phenotypically heterogeneous condition characterized by impairments in social interaction, communication, and the presence of repetitive behavior and restricted interests. It is a model syndrome to investigate neural interaction and integration at the nexus of language and social cognition. This paper considers the problems of language acquisition in AD from an evolutionary and ontogenetic context.

Regression in autistic spectrum disorders.

A significant proportion of children diagnosed with Autistic Spectrum Disorder experience a developmental regression characterized by a loss of previously-acquired skills. This may involve a loss of speech or social responsitivity, but often entails both. This paper critically reviews the phenomena of regression in autistic spectrum disorders, highlighting the characteristics of regression, age of onset, temporal course, and long-term outcome.

Speech perceived through a damaged temporal window: lessons from word deafness and aphasia.

Word deafness is an intriguing neurological syndrome characterized by severe difficulties in the ability to understand or reproduce spoken language with otherwise intact speech production and nonauditory language comprehension. The disorder is of significant theoretical importance because it putatively supports the modularity of speech recognition from more central language computational networks. However, the specificity and functional locus of the processing disturbances resulting in word deafness remains unclear.

Activation of human auditory cortex during speech perception: effects of monaural, binaural, and dichotic presentation.

We used a rapid event-related functional magnetic resonance imaging (fMRI) paradigm to compare cortical activation to speech tokens presented monaurally to each ear, binaurally, and dichotically. Two forms of dichotic conditions were examined: one presented consonant-vowel (CV) syllables simultaneously to each ear while the other paired a CV syllable with a non-speech stimulus (band-limited noise). Right-handed adults were asked to differentially respond to serially presented target and distractor CV syllables.

Etiologic subtypes of attention-deficit/hyperactivity disorder: brain imaging, molecular genetic and environmental factors and the dopamine hypothesis.

Multiple theories of Attention-Deficit/Hyper-activity Disorder (ADHD) have been proposed, but one that has stood the test of time is the dopamine deficit theory. We review the narrow literature from recent brain imaging and molecular genetic studies that has improved our understanding of the role of dopamine in manifestation of symptoms of ADHD, performance deficits on neuropsychological tasks, and response to stimulant medication that constitutes the most common treatment of this disorder. First, we consider evidence of the presence of dopamine deficits based on the recent literature that (1) confirms abnormalities in dopamine-modulated frontal-striatal circuits, reflected by size (smaller-than-average components) and function (hypoactivation); (2) clarifies the agonist effects of stimulant medication on dopaminergic mechanisms at the synaptic and circuit level of analysis; and (3) challenges the most-widely accepted ADHD-related neural abnormality in the dopamine system (higher-than-normal dopamine transporter [DAT] density).

Attention-deficit/hyperactivity disorder: a neuropsychological perspective towards DSM-V.

Neuropsychological methods and techniques have much to offer in the evaluation of the individual suspected as having Attention-Deficit/Hyperactivity Disorder (ADHD). After a review of the historical evolution of the ADHD concept, incidence and prevalence, and DSM-IV criteria for diagnosis, especially as regards omission related to gender differences, and other associated cultural, familial, socioenvironmental, and subject influences, this paper describes a number of dilemmas and obstacles encountered in clinical practice. Included are the confounds associated with the wide range of possible comorbidities, the insufficiency of current DSM-IV criteria, the emergence of subtype differentiation and its impact on diagnosis and treatment.

On pure word deafness, temporal processing, and the left hemisphere.

Pure word deafness (PWD) is a rare neurological syndrome characterized by severe difficulties in understanding and reproducing spoken language, with sparing of written language comprehension and speech production. The pathognomonic disturbance of auditory comprehension appears to be associated with a breakdown in processes involved in mapping auditory input to lexical representations of words, but the functional locus of this disturbance and the localization of the responsible lesion have long been disputed. We report here on a woman with PWD resulting from a circumscribed unilateral infarct involving the left superior temporal lobe who demonstrated significant problems processing transitional spectrotemporal cues in both speech and nonspeech sounds.

The effect of genetic differences and ovarian failure: intact cognitive function in adult women with premature ovarian failure versus turner syndrome.

Premature ovarian failure (POF) is generally defined as amenorrhea, hypoestrogenism, and elevated gonadotropins occurring in a woman before the age of 40 yr. Usually, the etiology is unknown. Turner syndrome (TS, monosomy X), also associated with ovarian failure, has a characteristic neurocognitive profile.

Acquired epileptiform aphasia: a dimensional view of Landau-Kleffner syndrome and the relation to regressive autistic spectrum disorders.

Acquired epileptiform aphasia (AEA) is characterized by deterioration in language in childhood associated with seizures or epileptiform electroencephalographic abnormalities. Despite an extensive literature, discrepancies and contradictions surround its definition and nosological boundaries. This paper reviews current conceptions of AEA and highlights variations in the aphasic disturbance, age of onset, epileptiform EEG abnormalities, temporal course, and long-term outcome.