This paper reviews the literature on the Nl wave of the human auditory evoked potential. It concludes that at least six different cerebral processes can contribute to (he negative wave recorded from the scalp with a peak latency between 50 and 150 ms: a component generated in the auditory-cortex on the supratemporal plane, a component generated in the association cortex on the lateral aspect of the temporal and parietal cortex, a component generated in the motor and premotor cortices, the mismatch negativity, a temporal component of the processing negativity, and a frontal component of the processing negativity, The first three, which can be considered ‘true’ N1 components, are controlled by the physical and temporal aspects of the stimulus and by the general state of the subject. The other three components are not necessarily elicited by a stimulus but depend on the conditions in which the stimulus occurs. They often last much longer than the true N1 components that they overlap.

The N1 wave of the human electric and magnetic response to sound: a review and an analysis of the component structure

This is a proposal of the Movement Disorder Society for a clinical classification of tremors. The classification is based on the distinction between rest, postural, simple kinetic, and intention tremor (tremor during target-directed movements). Additional data from a medical history and the results of a neurologic examination can be combined into one of the following clinical syndromes defined in this statement: enhanced physiologic tremor, classical essential tremor (ET), primary orthostatic tremor, task- and position-specific tremors, dystonic tremor, tremor in Parkinson's disease (PD), cerebellar tremor, Holmes' tremor, palatal tremor, drug-induced and toxic tremor, tremor in peripheral neuropathies, or psychogenic tremor. Conditions such as asterixis, epilepsia partialis continua, clonus, and rhythmic myoclonus can be misinterpreted as tremor. The features distinguishing these conditions from tremor are described. Controversial issues are outlined in a comment section for each item and thus reflect the open questions that at present cannot be answered on a scientific basis. We hope that this statement provides a basis for better communication among clinicians working in the field and stimulates tremor research.

Consensus statement of the Movement Disorder Society on tremor

An overview of the following six cortical zones that have been defined in the presurgical evaluation of candidates for epilepsy surgery is given: the symptomatogenic zone; the irritative zone; the seizure onset zone; the epileptogenic lesion; the epileptogenic zone; and the eloquent cortex. The stepwise historical evolution of these different zones is described. The current diagnostic techniques used in the definition of these cortical zones, such as video-EEG monitoring, MRI and ictal single photon emission computed tomography, are discussed. Established diagnostic tests are set apart from procedures that should still be regarded as experimental, such as magnetoencephalography, dipole source localization and spike-triggered functional MRI. Possible future developments that might lead to a more direct definition of the epileptogenic zone are presented.

/pdf/presurgical-evaluation-of-epilepsy-216chlpgp2.pdf

Presurgical evaluation of epilepsy.

We performed a comprehensive cognitive, neuroimaging, and genetic study of 31 patients with primary progressive aphasia (PPA), a decline in language functions that remains isolated for at least 2 years. Detailed speech and language evaluation was used to identify three different clinical variants: nonfluent progressive aphasia (NFPA; n = 11), semantic dementia (SD; n = 10), and a third variant termed logopenic progressive aphasia (LPA; n = 10). Voxel-based morphometry (VBM) on MRIs showed that, when all 31 PPA patients were analyzed together, the left perisylvian region and the anterior temporal lobes were atrophied. However, when each clinical variant was considered separately, distinctive patterns emerged: (1) NFPA, characterized by apraxia of speech and deficits in processing complex syntax, was associated with left inferior frontal and insular atrophy; (2) SD, characterized by fluent speech and semantic memory deficits, was associated with anterior temporal damage; and (3) LPA, characterized by slow speech and impaired syntactic comprehension and naming, showed atrophy in the left posterior temporal cortex and inferior parietal lobule. Apolipoprotein E e4 haplotype frequency was 20% in NFPA, 0% in SD, and 67% in LPA. Cognitive, genetic, and anatomical features indicate that different PPA clinical variants may correspond to different underlying pathological processes.

/pdf/cognition-and-anatomy-in-three-variants-of-primary-1yinnydoyf.pdf

Cognition and Anatomy in Three Variants of Primary Progressive Aphasia

HUMAN speech requires complex planning and coordination of mouth and tongue movements. Certain types of brain injury can lead to a condition known as apraxia of speech, in which patients are impaired in their ability to coordinate speech movements but their ability to perceive speech sounds, including their own errors, is unaffected1,3. The brain regions involved in coordinating speech, however, remain largely unknown. In this study, brain lesions of 25 stroke patients with a disorder in the motor planning of articulatory movements were compared with lesions of 19 patients without such deficits. A robust double dissociation was found between these two groups. All patients with articulatory planning deficits had lesions that included a discrete region of the left precentral gyms of the insula, a cortical area beneath the frontal and temporal lobes. This area was completely spared in all patients without these articulation deficits. Thus this area seems to be specialized for the motor planning of speech.

A new brain region for coordinating speech articulation

Motor Speech Disorders

Thirty-second speech samples were studied of at least 30 patients in each of 7 discrete neurologic groups, each patient unequivocally diagnosed as being a representative of his diagnostic group. Three judges independently rated each of these samples on each of 38 dimensions of speech and voice using a 7-point scale of severity. Computer analysis based on the means of the three ratings on each patient on each dimension yielded results leading to these conclusions: (1) Speech indeed follows neuroanatomy and neurophysiology. There are multiple types or patterns of dysarthria, each mirroring a different kind of abnormality of motor functioning. (2) These patterns of dysarthria can be differentiated; they sound different. They consist of definitive groupings of certain dimensions of speech and voice, deviant to distinctive degrees. (3) Five types of dysarthria were delineated: flaccid dysarthria (in bulbar palsy), spastic dysarthria (in pseudobulbar palsy), ataxic dysarthria (in cerebellar disorders), hypokine...

Differential Diagnostic Patterns of Dysarthria

Correlation matrices were used to demonstrate co-occurrence of deviant speech dimensions observed. Application of this technique led to the emergence of eight distinctive clusters of dysfunction. Each of seven neurologic disorders studied had its own unique group or pattern of clusters. However, any one cluster emerged in and was shared by more than one disorder. Inspection of the dimensions present in a cluster permitted giving the cluster a logically determined name based usually on the defective physiology responsible for the cluster. Knowledge of the neuromuscular characteristics of each disorder led to deductions concerning the neuromuscular substrate for each cluster. Intercluster correlations yielded clues concerning co-occurrence of certain neuromuscular defects. Further inspection led to identification of the probable neuromuscular bases of individual deviant speech dimensions. These conclusions may serve as hypotheses for more accurate physiologic and neurophysiologic measurements to further del...

Clusters of deviant speech dimensions in the dysarthrias.

The epidemiology of generalized and focal dystonias was investigated in the Rochester, Minnesota, population over the period 1950-1982. The crude incidence of generalized dystonia was 2 per million persons per year, and for all focal dystonias combined, 24 per million per year. The crude prevalence rate was 34 per million persons for generalized dystonia and 295 per million persons for all focal dystonias. Torticollis was the most common focal dystonia; essential blepharospasm, oromandibular dystonia, spasmodic dysphonia, and writer's cramp were less common and had roughly equal incidence and prevalence rates.

Epidemiology of focal and generalized dystonia in Rochester, Minnesota.

The treatment of adductor spasmodic dysphonia using botulinum toxin A was conducted in 13 patients as a double-blind, placebo-controlled study. Patients were diagnosed independently by an interdisciplinary team consisting of speech pathologists, an otolaryngologist, and a neurologist. The toxin or saline was injected into each thyroarytenoid muscle under electromyographic and laryngoscopic guidance. Botulinum toxin A markedly reduced perturbation, decreased fundamental frequency range, and improved the spectrographic characteristics of the voice. Fundamental frequency and phonation time remained unchanged. Patients injected with botulinum toxin A noticed significant improvement in their voices in comparison with the placebo-treated group. Excessive breathiness of the voice occurred in two patients, and mild bleeding in one patient in the botulinum toxin A-treated group. Injection with saline resulted in edema of the vocal cord in one patient. Botulinum toxin A proved to be an effective and safe treatment of adductor spasmodic dysphonia.

Arnold E. Aronson

Papers

Motor Speech Disorders

Differential Diagnostic Patterns of Dysarthria

Clusters of deviant speech dimensions in the dysarthrias.

Epidemiology of focal and generalized dystonia in Rochester, Minnesota.

Double-blind controlled study of botulinum toxin in adductor spasmodic dysphonia