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PubMed2001

Selective impairment of verb processing associated with pathological changes in Brodmann areas 44 and 45 in the motor neurone disease-dementia-aphasia syndrome.

We report six patients with clinically diagnosed and electrophysiologically confirmed motor neurone disease (MND), in whom communication problems were an early and dominant feature. All patients developed a progressive non-fluent aphasia culminating in some cases in complete mutism. In five cases, formal testing revealed deficits in syntactic comprehension. Comprehension and production of verbs were consistently more affected those that of nouns and this effect remained stable upon subsequent testing, despite overall deterioration. The classical signs of MND, including wasting, fasciculations and severe bulbar symptoms, occurred over the following 6-12 months. The behavioural symptoms ranged from mild anosognosia to personality change implicating frontal-lobe dementia. In three cases, post-mortem examination has confirmed the clinical diagnosis of MND-dementia. In addition to the typical involvement of motor and premotor cortex, particularly pronounced pathological changes were observed in the Brodmann areas 44 (Broca's area) and 45. The finding of a selective impairment of verb/action processing in association with the dementia/aphasia syndrome of MND suggests that the neural substrate underlying verb representation is strongly connected to anterior cortical motor systems.

Aged↗
PubMed2003

Neural resources for processing language and environmental sounds: evidence from aphasia.

Although aphasia is often characterized as a selective impairment in language function, left hemisphere lesions may cause impairments in semantic processing of auditory information, not only in verbal but also in nonverbal domains. We assessed the 'online' relationship between verbal and nonverbal auditory processing by examining the ability of 30 left hemisphere-damaged aphasic patients to match environmental sounds and linguistic phrases to corresponding pictures. The verbal and nonverbal task components were matched carefully through a norming study; 21 age-matched controls and five right hemisphere-damaged patients were also tested to provide further reference points. We found that, while the aphasic groups were impaired relative to normal controls, they were impaired to the same extent in both domains, with accuracy and reaction time for verbal and nonverbal trials revealing unusually high correlations (r = 0.74 for accuracy, r = 0.95 for reaction time). Severely aphasic patients tended to perform worse in both domains, but lesion size did not correlate with performance. Lesion overlay analysis indicated that damage to posterior regions in the left middle and superior temporal gyri and to the inferior parietal lobe was a predictor of deficits in processing for both speech and environmental sounds. The lesion mapping and further statistical assessments reliably revealed a posterior superior temporal region (Wernicke's area, traditionally considered a language-specific region) as being differentially more important for processing nonverbal sounds compared with verbal sounds. These results suggest that, in most cases, processing of meaningful verbal and nonverbal auditory information break down together in stroke and that subsequent recovery of function applies to both domains. This suggests that language shares neural resources with those used for processing information in other domains.

Adult↗
PubMed2003

Revisiting the dissociation between singing and speaking in expressive aphasia.

We investigated the production of sung and spoken utterances in a non-fluent patient, C.C., who had a severe expressive aphasia following a right-hemisphere stroke, but whose language comprehension and memory were relatively preserved. In experiment 1, C.C. repeated familiar song excerpts under four different conditions: spoken lyrics, sung lyrics on original melody, lyrics sung on new but familiar melody and melody sung to a neutral syllable "la". In experiment 2, C.C. repeated novel song excerpts under three different conditions: spoken lyrics, sung lyrics and sung-to-la melody. The mean number of words produced under the spoken and sung conditions did not differ significantly in either experiment. The mean number of notes produced was not different either in the sung-to-la and sung conditions, but was higher than the words produced, hence showing a dissociation between C.C.'s musical and verbal productions. Therefore, our findings do not support the claim that singing helps word production in non-fluent aphasic patients. Rather, they are consistent with the idea that verbal production, be it sung or spoken, result from the operation of same mechanisms.

Aphasia, Broca↗
PubMed2006

Progranulin gene mutations associated with frontotemporal dementia and progressive non-fluent aphasia.

Frontotemporal lobar degeneration (FTLD) refers to a focal, non-Alzheimer form of cerebral degeneration that encompasses the distinct clinical syndromes of frontotemporal dementia (FTD), progressive non-fluent aphasia (PNFA) and semantic dementia. Some patients show tau-based pathological changes and in familial cases mutations have been identified in the microtubule-associated protein tau gene (MAPT) on chromosome 17q21. However, many cases are tau-negative, showing instead ubiquitin-immunoreactive (UBQ-ir) neuronal cytoplasmic inclusions and neurites, and in some familial cases UBQ-ir neuronal intranuclear inclusions of a lentiform appearance. Very recently, mutations have been identified in familial cases in the progranulin (PGRN) gene, also on chromosome 17q21. Clinical, pathological and molecular diversity within FTLD highlights the importance of careful examination of clinical-pathological-genetic relationships. This paper reports, for the first time, a clinico-pathological investigation of two FTLD families with PGRN mutations, and compares the clinical characteristics with those of patients studied in the department with MAPT mutations. The clinical profile associated with PGRN mutations constituted, in some patients, a prototypical picture of FTD and in others one of PNFA, both profiles occurring within the same family. Patients with PGRN mutations exhibited phonological deficits, whereas in patients with MAPT mutations language abnormalities, when present in addition to the prominent behavioural disorder, take the form of semantic disturbance. The findings provide compelling evidence for the link between FTD and PNFA, while raising the possibility of identifiable clinical differences between FTLD patients with MAPT and PGRN mutations.

Aged↗
PubMed1993

Hyperemic receptive aphasia on neuroSPECT.

NeuroSPECT of regional cerebral blood flow (rCBF) with Tc-99m HMPAO demonstrated left temporoparietal hyperemia in two patients with acute receptive aphasia. This finding prompted further testing with electroencephalography that added to the impression of ictal dysphasia. The differential diagnosis in one case included complicated migraine. NeuroSPECT depicts blood flow abnormalities in acute aphasic disorders, either due to ischemia, which is most commonly the cause, or due to hyperemia secondary to migraine or epilepsy. The treatment and prognosis of these latter conditions differ from stroke, and thus SPECT plays a role in patient management.

Adult↗
PubMed2003

Pick Complex: an integrative approach to frontotemporal dementia: primary progressive aphasia, corticobasal degeneration, and progressive supranuclear palsy.

BACKGROUND: Frontotemporal dementia (FTD) is a new label for clinical Pick's disease (PiD) because the eponymic term is increasingly restricted to the pathologic finding of Pick bodies. This restriction created the impression that PiD is rare and that is it difficult to diagnose. FTD is also a term most often used for behavioral and personality alterations. Primary progressive aphasia (PPA) and corticobasal degeneration (CBD), formerly the extrapyramidal variety of PiD, are also part of the syndrome. Recently, chromosome 17 localization and tau mutations were discovered in familial forms of the disease. REVIEW SUMMARY: FTD consists of behavioral and personality changes, often beginning with apathy and disinterest, which may be mistaken for depression. Disinhibition and perseverative, compulsive behavior often appear at the same time. A quantifiable frontal behavioral inventory is useful in the diagnosis beyond a checklist. The second type of presentation is progressive language loss (PPA). A less common variety is semantic dementia: the meaning of nouns and objects is lost. As the disease progresses, all components tend to overlap. CBD and progressive supranuclear palsy (PSP), although described as distinct entities, show a great deal of clinical, pathologic, genetic, and biochemical overlap. The evidence suggests they also belong to the complex. The association of motor neuron disease (MND) with FTD and other varieties of the complex is also reviewed. CONCLUSIONS: Clinical Pick's disease or Pick Complex includes the overlapping syndromes of FTD, PPA, CBD, PSP, and FTD-MND. The neuropathological and genetic spectrum should be viewed with emphasis on the commonalities rather than the differences, allowing the recognition of the relatively high frequency of this presenile syndrome.

Aphasia, Primary Progressive↗
PubMed2006

Mechanisms of recovery in stroke patients with hemiparesis or aphasia: new insights, old questions and the meaning of therapies.

PURPOSE OF REVIEW: The mechanisms responsible for recovery after stroke in patients with hemiparesis or aphasia are under intense study, since knowledge of these mechanisms is a prerequisite for choosing which therapy a patient receives and when to apply it. RECENT FINDINGS: Most of the recent insights are obtained with longitudinal studies using functional imaging and direct cortical stimulation during the process of recovery. They reveal that reorganization is a highly dynamic process, involving the establishment of new communications in the remaining system and showing similarities to learning processes in healthy individuals. Lesion localization is a major determinant for recovery and the pattern of reorganization. Neurobiological hypotheses lead to clinical studies, which in turn are now used to confirm or reject these hypotheses. SUMMARY: Although our understanding of the mechanisms responsible for recovery is increasing, the application of this knowledge in daily praxis is still limited. A better understanding of the underlying mechanisms, however, can lead to appropriate therapies for individual patients.

Aphasia↗
PubMed1989

Psychological responses in aphasia: theoretical considerations and nursing implications.

Patients experiencing aphasia from brain damage due to cerebrovascular accident or craniocerebral trauma manifest psychological and behavioral changes resulting from communication deficits. Although literature is controversial regarding the etiology of the psychological responses, the major determinant of these responses depends upon the premorbid personality. Specific nursing diagnoses focusing on communication, knowledge deficits and grieving are presented with suggested interventions.

Aphasia↗
PubMed1994

Impairments of speech production and speech perception in aphasia.

The basis of speech production and speech perception deficits in aphasia relates to implementation and access rather than to the underlying representation or knowledge base of the sound structure of language. Speech production deficits occur on the phonological level in which the incorrect phonological form of the word is selected but is implemented correctly, and the phonetic level in which the correct sound segments are selected but articulatory implementation is impaired. Phonological deficits emerge regardless of lesion site, whereas phonetic deficits have a specific localized neuroanatomical substrate. Phonetic deficits are not linguistic but affect particular articulatory movements. Speech perception impairments emerge in nearly all aphasic patients, suggesting that the neural basis for speech perception is broadly distributed in the language hemisphere. The impairment reflects the misperception of phonetic features rather than a deficit in the auditory processing of speech and emerges particularly as the sound properties of speech contact the lexicon.

Aphasia↗
PubMed2006

Different patterns of Mini Mental Status Examination responses in primary progressive aphasia and Alzheimer's disease.

Primary progressive aphasia (PPA) syndrome is frequently misdiagnosed--particularly in favour of Alzheimer's disease (AD). Misdiagnosis is related to the heterogeneity of language disorders at onset, variability in the rate of clinical progression and the low prevalence of PPA syndrome, compared with AD. The aim of this study was to determine whether a patient's first Mini Mental Status Examination (MMSE) might provide insight into differentiating between PPA and AD. We compared item scores for the first, complete MMSE in consecutive patients with PPA versus matched patients with AD. Word recall and constructional praxis were significantly better in patients subsequently diagnosed as suffering from PPA. Patients with AD performed significantly better in terms of word registration, object naming, repetition and verbal direction. Our findings indicate that the various MMSE item scores may be helpful in differentiating PPA and AD in the first few years of the disease.

Aged↗
PubMed1982

Temporary regression during language acquisition: a linguistic analysis of a 2 1/2-year-old child with epileptic aphasia.

A 2 1/2-year-old child suffered a brief and temporary regression of speech, with complete recovery, on two separate occasions. During the second episode, clinical evaluation and electroencephalographic data led to the diagnosis of epileptic aphasia. The child's conversations at home were tape-recorded by the mother during the acute phase of the disorder and the recovery period, and transcripts of the conversations were analysed by means of a linguistic profile. These analyses confirmed the clinical improvement in the child's language abilities, which occurred within a few days. This case illustrates the remarkably specialised and localised cerebral function which underlies speech production at an early age. It indicates that this function can be interfered with selectively and temporarily, without any significant effect on general behaviour.

Aphasia↗
PubMed1985

On the positive semiology of acquired aphasia in children.

Eleven unselected cases of aphasia in children seen over a three-year period in a neuropaediatric centre are reported. Contrary to current reports in the literature, the children disclosed a semiology similar to that of adults, particularly for frequency and distribution of 'positive signs' such as paraphasias. The reasons for this discrepancy are discussed and an hypothesis of early intrahemispheric specialization is proposed.

Adolescent↗
PubMed1985

Age of onset and outcome in 'acquired aphasia with convulsive disorder' (Landau-Kleffner syndrome).

The relationship between age at onset of language disorder and eventual outcome was examined in 45 cases of Landau-Kleffner syndrome reported in the literature, all of whom had been followed up to at least 12 years of age. A strong relationship was found, which is opposite to that for childhood aphasia after structural lesions of the left hemisphere; i.e. in Landau-Kleffner syndrome, the older the child at onset the better the prognosis for language.

Age Factors↗
PubMedDate not supplied

Paroxysmal aphasias.

Forty cases of paroxysmal aphasia were found in a sample of 4,000 patients with epilepsy. Twenty-five had structural brain damage demonstrated by CT scan. Except for two cases, the epileptic focus was located in the left hemisphere. Two patients had a paroxysmal alexia associated with the aphasic disorder. Though preliminary, our data suggest a correlation of dysphasic components with posterior lesions and phonatory components with anterior lesions.

Adolescent↗
PubMedDate not supplied

Acquired epileptic aphasia (the Landau-Kleffner syndrome) due to neurocysticercosis.

A healthy 7-year-old boy developed a language disorder associated with clinical seizures and a paroxysmal EEG. Computed tomography and magnetic resonance imaging revealed a small cysticercus deep in the left Sylvian fissure. We postulate a cause and effect relationship between the parasitic cyst and the clinical manifestations supported by the strategic location of the cyst and the critical age at which this lesion developed. This case provides evidence that the syndrome of acquired epileptic aphasia may be explained in terms of an unilateral structural brain lesion.

Aphasia↗
PubMed1980

A very short version of the Minnesota Aphasia Test.

A very short version of the Minnesota Differential Diagnosis of Aphasia Test is described. It is standardized on 86 aphasics and reduces the original 43 subtests to just four; takes less than 15 minutes to give; correlates by over 0.9 with the total full test; and misclassified only 13 per cent of subjects. These four subtests are identifying objects named serially (A4), oral reading of words (B8), naming pictures (C13) and written spelling (D6). This extensive shortening is made feasible by the high degree of redundancy in the original full version. The test is designed to detect asphasia in general, and not to partition aphasics typologically, for which the full Minnesota is required.

Aphasia↗