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[Clinical aspects of frontotemporal dementia].

Frontotemporal neurodegeneration can cause three typical clinical syndromes: frontotemporal dementia (FTD), primary progressive aphasia (PPA) and semantic dementia (SD). In the present paper we review these syndromes, highlighting FTD. Four case examples are presented. At the early stage of FTD changes of personality and social conduct are prominent, whereas cognitive functions are relatively well preserved. Since the usual dementia tests are not sufficiently sensitive to disclose non-cognitive symptoms, clinical diagnosis as well as differentiation from non-organic psychiatric disorders can be difficult. Detailed history, thorough clinical examination, and neuropsychological testing are required to establish the diagnosis. EEG and functional brain imaging may be helpful. The choice of therapeutic options for FTD is extremely limited. Medications may be used to treat neuropsychiatric symptoms. There is little experience with non-pharmacologic behaviour modification and milieu treatment approaches. The problems that FTD imposes on caregivers are dissimilar to those arising from Alzheimer's disease. Families receive little or no support so that early nursing home admission of patients is common.

Dementia↗

Persistent preceding focal neurologic deficits in children with chronic Epstein-Barr virus encephalitis.

Epstein-Barr virus encephalitis is a self-limiting disease with few sequelae. Persistence of neurologic deficits prior to and after the acute illness has yet to be described in children. We describe five children with persistent cognitive and focal neurologic deficits due to chronic Epstein-Barr virus encephalitis with various T2-weighted magnetic resonance imaging abnormalities. Clinical features were a 9-year-old boy with aphasia and apraxia, an 11-year-old girl with impulsivity and inappropriate behavior, a 17-year-old boy with deterioration of cognitive skills and judgment, a 5-year-old boy with complex-partial seizures, and a 6-year-old girl with obsessive-compulsive behavior. All patients had elevated serum Epstein-Barr virus titers for acute infection, with cerebrospinal fluid polymerase chain reaction positive for Epstein-Barr virus in four patients. Three children were treated with methylprednisolone with minimal improvement without changes on magnetic resonance imaging. Epstein-Barr virus encephalitis can present with chronic and insidious neurologic symptoms and should be considered in the differential diagnosis of children with acute or chronic neurologic illness of unknown etiology.

Adolescent↗

Different patterns of magnetic resonance imaging atrophy for frontotemporal lobar degeneration syndromes.

BACKGROUND: Frontotemporal lobar degeneration (FTLD) is an uncommon degenerative dementia that presents with focal cognitive and behavioral deficits. OBJECTIVE: To determine the correlation of the different presentations of FTLD with structural neuroimaging findings. DESIGN AND PATIENTS: In a blinded study, we retrospectively evaluated the clinical presentations and magnetic resonance imaging (MRI) patterns of atrophy in 59 patients with FTLD and 26 patients with probable Alzheimer disease at a memory disorders clinic. RESULTS: Analysis of variance revealed a significant difference in the patterns of atrophy in the FTLD and Alzheimer disease groups. Patients with FTLD presenting with altered personal conduct had significant bifrontal atrophy, whereas patients presenting with semantic dementia had significant left temporal and bifrontal atrophy compared with other groups. Disinhibited behavior and hyperphagia correlated with right frontal atrophy, and fluent, anomic aphasia correlated with left temporal atrophy. CONCLUSIONS: We found that the type of clinical presentation of FTLD correlates with specific areas of atrophy. Our method of analysis may be useful to elicit further anatomic-behavioral relationships in degenerative brain disorders.

Aged↗

[Magnetic stimulation of the scalp: the responses of the masseter muscles. II].

Magnetic stimulation of nervous elements is a new technique of investigation in intact alert man. Electromyographic responses of the masseter muscles were recorded in normal volunteers and in two patients with hemispheric or capsular lesions, both by surface and needle electrodes. In the patients "cortical" responses were absent when stimulating the affected hemisphere and present when stimulating the unaffected one. These findings suggest that the direct facilitating cortico-nuclear projections for the masseter muscle are mainly crossed. Nevertheless the presence of clear ipsilateral responses after stimulation of the unaffected hemisphere demonstrates the existence of uncrossed projections; in fact only the ipsilateral motor cortex can be the site of origin of the responses in these patients.

Adult↗

Chronic colchicine-induced myopathy and neuropathy.

The presented case concerns a 77-year old man who had been chronically taking colchicine for treatment of gout. He was admitted because of a transient ischemic cerebrovascular attack with motor aphasia and complained of preexisting paraesthesias in the lower extremities. Neurological examination revealed a global muscular weakness, absent myotatic reflexes and a diminished sensation. Serum creatine kinase (CK) levels were increased and electromyography showed spontaneous fibrillations in deltoid muscles, positive spikewaves in deltoids and anterior tibial muscles. Motor and sensory conduction velocities were mildly reduced. Nerve biopsy findings were compatible with a chronic axonal neuropathy having produced a significant loss of myelinated axons and also denervation features of unmyelinated axons. In muscle, combined neurogenic and myogenic features were found. The former result from the axonal neuropathy. The latter were mainly characterized by focal myofibrillar disorganisation and accumulation of autophagic vacuoles in muscle fibres. The presented neuromuscular symptoms and signs, the increased CK values, the electromyographic and nerve conduction velocity findings as well as nerve and muscle biopsy observations, are consistent with the diagnosis of colchicine-induced myopathy and neuropathy. Furthermore, the disappearance of paraesthesias, normalisation of CK values, and disappearance of fibrillations and positive spike waves in deltoid and anterior tibial muscles on electromyography, after stopping of the colchicine therapy, supported the diagnosis.

Aged↗

Phonetic disintegration in aphasia: acoustic analysis of spectral characteristics for place of articulation.

In this study, we attempted to determine whether phonetic disintegration of speech in Broca's aphasia affects the spectral characteristics of speech sounds as has been shown for the temporal characteristics of speech. To this end, we investigated the production of place of articulation in Broca's aphasics. Acoustic analysis of the spectral characteristics for stop consonants were conducted. Results indicated that the static aspects of speech production were preserved, as Broca's aphasics seemed to be able to reach the articulatory configuration for the appropriate place of articulation. However, the dynamic aspects of speech production seemed to be impaired, as their productions reflected problems with the source characteristics of speech sounds and with the integration of articulatory movements in the vocal tract. Listener perceptions of the aphasics' productions were compared with acoustic analyses for these same productions. The two measures were related; that is, the spectral characteristics of the utterances provided salient cues for place of articulation perception. An analysis of the occurrences of errors along the dimensions of voicing and place showed that aphasics rarely produce utterances containing both voice and place substitutions.

Adult↗

[Aphasia and parietal syndrome as the presenting symptoms of a demyelinating disease with pseudotumoral lesions].

INTRODUCTION: Multiple sclerosis (MS) often presents with sensory symptoms, which are usually due to spinothalamic or spinal cord disorders; parietal syndrome is, however, very rare as the initial symptom. Likewise, aphasia is also an infrequent symptom of MS; in the few cases that have been reported, it is usually linked to the existence of important pseudotumoral lesions. CASE REPORT: We describe the case of a 31-year-old female with a 48-hour history of a progressive clinical picture consisting in nominal aphasia and a sensory parietal syndrome. Magnetic resonance imaging showed a lesion 3.6 cm in diameter that was hyperintense in T2 with perilesional edema and minimal gadolinium uptake, along with other images that revealed increased signal intensity in the periventricular subcortical white matter on the right-hand side and in the left-hand frontal subcortical region. A spectroscopic analysis of the largest lesion revealed that this lesion showed evidence of inflammation, with cell destruction and replacement, although it was not possible to distinguish between a demyelinating disease and a high grade glioma. Hence, a brain biopsy was required in order to reach the final diagnosis of demyelinating pseudotumoral lesion. CONCLUSIONS: Giant pseudotumoral plaques are a rare form of presenting symptom in MS; use of the clinical features, simple images and spectroscopy is not a very reliable means of reaching a differential diagnosis with a tumour and this often makes it necessary to conduct a biopsy study of the lesion.

Adult↗

[Use of discourse in neuropsychological examinations].

The study was conducted to establish the ability of aphatics and patients with lesions in the right cerebral hemisphere to reconstruct the superstructure of narrative discourse and procedural discourse. It was found that despite evident speech disturbances the patients with aphasia maintained the ability of correct reconstruction of the basic elements of a discourse, while patients with right hemisphere lesions had difficulties in correct construction of the structure of the text evidencing specific disturbances of discourse.

Adolescent↗

Dissociations among functional categories in Korean agrammatism.

This study investigated the hypothesis that the syntactic trees formed by individuals with agrammatic aphasia cannot be constructed any higher than an impaired node as suggested by the tree-pruning hypothesis (Friedman, 1994; Friedmann & Grodzinsky, 1997) and hypothesis. It also examined their following implication that the members of a certain functional category are subject to the same degree of impairment. Two experiments were conducted to investigate a Korean agrammatic patient's use and understanding of three functional categories--Mood, Tense, and Complementizer. The results showed a dissociation among functional categories that preserves the higher node while leaving the lower node impaired both in production and comprehension. Another dissociation was found among members of the same category depending on their linear position in the clause. These results contrast with the predictions of the tree-pruning hypothesis, suggesting that the nearer to the end of the clause a functional element is located, the better it is preserved in Korean agrammatism.

Aphasia, Broca↗

The problem of generalizing to a language population: a "random" controversy.

The rationale behind the use of item statistics as well as objections to their use are reviewed. It is argued that investigators conducting language research with disordered populations should follow the example of those conducting language research with normal populations and consider calculating item statistics if they wish their results to generalize beyond the specific items tested.

Aphasia↗

Language and speech defects.

A review of recent research conducted at the Rusk Institute of Rehabilitation Medicine in New York City concerning speech and language deficits in closed head injured patients (CHI). One hundred and twenty-five consecutive admissions of 125 closed head injured post coma patients were administered standardized aphasia tests to determine the presence and nature of verbal deficits. Mean time since injury for the group was 45 weeks. All patients, without exception, evidenced linguistic impairment. The population fell into three relatively equally sized groups: classic aphasia, dysarthria accompanied by linguistic deficits, and "subclinical" aphasic deficits. The study results suggest that linguistic functions are particularly vulnerable in severe head injury. A second study compared aphasia secondary to CVA and CHI. When age and time since onset were controlled, aphasic CHI and CVA patients are more similar than different in linguistic task performance and overall functional communication effectiveness. The results suggest that the therapeutic approaches traditionally implemented with CVA aphasic patients are appropriate for the management of CHI aphasic patients as well.

Adolescent↗

Phonologically related lexical repetition disorder: a case study.

Errors of repetition in aphasia are most often nonword substitutions. Phonologically related lexical errors, or formal errors, are real-word substitutions that overlap with target words in sound. In the present research we present the case of an aphasic patient, MMB, who produced an unusually high rate of formal paraphasias in repetition. Six experiments were conducted to investigate the combination of impairments contributing to MMB's pattern of repetition and to test the predictions made by two theories of formal errors. MMB's formal errors in repetition were influenced by target frequency, but not by target length or imageability. Formal errors tended to be more frequent than their targets and showed greatest phonological overlap with targets at initial consonant. These findings provided partial support for Martin and Saffran's fully interactive spreading activation account of formal errors and did not support Blanken's phonological interactive encoding account. In Experiment 6, the effect on repetition of increasing auditory verbal short-term memory (AVSTM) demands was examined using a paired word repetition experiment. Under these conditions, MMB produced semantic paraphasias for the first time, providing strong support for the Martin-Saffran hypothesis that phonologically related, and semantic, lexical repetition disorders lie on a continuum of severity moderated by the degree of AVSTM impairment.

Aphasia↗

Relation between cognitive function and mortality in middle-aged adults: the atherosclerosis risk in communities study.

An independent, inverse association between cognitive function and all-cause mortality has been reported in elderly cohorts. The purpose of this study was to determine whether the same association exists in middle-aged persons. The Atherosclerosis Risk in Communities Study is a cohort study initiated in 1987 to investigate the development of atherosclerosis in middle-aged persons. Three cognitive function measures were included in the second cohort examination conducted from 1990 to 1992 when the participants were aged 48-67 years: the Delayed Word Recall Test (DWRT), the Digit Symbol Substitution Test (DSST) (a subtest from the Wechsler Adult Intelligence Scale-Revised), and the Word Fluency Test from the Multilingual Aphasia Examination. Cox proportional hazards modeling was used to determine whether all-cause mortality ascertained through 1997 was associated with each measure after adjustment for sociodemographic, biologic, psychologic, and behavioral risk factors. Without adjustment, there was a significantly lower mortality hazard associated with higher scores on all three measures. After covariate adjustment, the hazard ratios for the DWRT and the DSST remained significant (hazard ratio1-point DWRT score increment = 0.90, 95% confidence interval: 0.84, 0.97; hazard ratio 7-point DSST score increment = 0.86, 95% confidence interval: 0.80, 0.93). Cognitive function measured in middle age appears to have prognostic importance for life expectancy similar to that reported in elderly adults.

Aged↗

Change in perfusion in acute nondominant hemisphere stroke may be better estimated by tests of hemispatial neglect than by the National Institutes of Health Stroke Scale.

BACKGROUND AND PURPOSE: It has been reported that National Institutes of Health Stroke Scale (NIHSS) scores correlate poorly with hypoperfused tissue measured by perfusion-weighted imaging (PWI) in nondominant hemisphere stroke. We conducted 2 studies to determine whether tests of hemispatial neglect provide a better measure of hypoperfusion and reperfusion than NIHSS in nondominant hemisphere stroke. METHODS: In study 1, 74 patients with acute ischemic, supratentorial stroke were administered the NIHSS, tests of neglect or aphasia, and diffusion-weighted imaging (DWI) and PWI on day 1 (<24 hours from onset) of stroke. Pearson correlations between volumes of PWI/DWI abnormality and functional tests were calculated. In study 2, 10 patients with acute, nondominant hemisphere stroke who were candidates for intervention to restore perfusion underwent PWI, DWI, NIHSS, and a line cancellation test on days 1 and 3. Correlations between change in volumes of PWI/DWI abnormality and change in functional tests were calculated. RESULTS: In study 1, in nondominant hemisphere stroke, volume of PWI abnormality correlated significantly with neglect scores (r=0.71; P<0.002) but not with NIHSS scores (r=0.39; P=NS). In dominant hemisphere stroke, volume of PWI abnormality correlated better with aphasia scores (r=0.50; P=0.0001) than with NIHSS scores (r=0.45; P=0.001). In study 2, change in volume of hypoperfused tissue on PWI correlated with change in line cancellation performance (r=0.83; P=0.003) but not with change in NIHSS score (r=0.26; P=NS). CONCLUSIONS: Tests of hemispatial neglect may better reflect dysfunction and reperfusion than NIHSS for patients with nondominant hemisphere stroke.

Acute Disease↗

Selective preservation of a lexical category in aphasia: dissociations in comprehension of body parts and geographical place names following focal brain lesion.

The selective dissociation of memory for proper names is discussed in the context of a review of dissociations involving broadly defined vs narrowly defined semantic categories in brain damaged adults. Two narrowly defined categories for which dissociations have been reported are body parts (selectively impaired in comprehension) and geographical place names (selectively preserved in comprehension). In this study, 167 aphasic patients were tested for their ability to correctly identify body parts and map locations from spoken names, relative to a baseline for correct responses on general word discrimination (object identification). Wernicke's aphasics and Global aphasics both had significantly more success in pointing to named map locations and significantly worse performance in pointing to named body parts than they had in selecting other objects from multiple choice. Anomic aphasics showed precisely the opposite pattern of results. Other aphasic subgroups (Broca's aphasics, Mixed Nonfluent aphasics, Conduction aphasics, Transcortical Motor and Transcortical Sensory aphasics, and Mixed Fluent aphasics) did not show significant deviations in either direction. We suggest that the selective preservation for place names may be related to their status as proper names.

Adult↗

Comprehension strategies determining reference in aphasia: a study of reflexivization.

This study explored the comprehension of reference in aphasia as reflected in reflexivization. A total of 132 test sentences were used consisting of two basic syntactic frames varying in the minimal distance principle and in the type of cues available in the sentence (syntax, syntax and morphology, syntax and lexical, and syntax, morphology, and lexical) for determining pronominal reference. Fifteen patients, five Broca's, five Conduction, and five Wernicke's, were asked to point to the picture (of two) corresponding to the auditorily presented test sentence. Results indicated that aphasic patients as a whole comprehend reference; however, their performance varied depending on the nature of the cues available in the sentence. All groups were impaired when only syntactic cues were available and showed a significant decrement in performance when the test sentence violated the minimal distance principle. As additional cues other than syntactic structure were provided, performance level increased. All groups, including the Broca's aphasics, showed only slight improvement with the presence of morphological cues. Wernicke's aphasic's performance was particularly vulnerable when lexical cues were added to syntactic cues. These results suggest that evaluation of a patient's deficit in sentence comprehension requires analysis of those attributes of the sentence contributing to its overall semantic interpretation.

Adult↗

Comprehending a word: the influence of speed and redundancy on auditory comprehension in aphasia.

To assess the contribution of various factors to comprehension of a target word, an auditory comprehension test was devised and administered to a series of aphasic patients. Patients were required to select from an array that picture which corresponded to a spoken target word. Each patient heard the target word under five conditions: (1) target spoken alone; (2) target embedded in a neutral (low redundancy) sentence enunciated at a normal rate of speaking; (3) target in a neutral sentence enunciated at a slow rate of speaking; (4) target embedded in a sentence containing semantic support (high redundancy); (5) target embedded in a sentence containing a semantically-deceptive element. Semantic redundancy and rate of presentation made contributions to comprehensibility. Semantic confusions were prevalent among all aphasics but, contrary to earlier reports, posterior patients were especially prone to acoustic confusions. Except for conduction aphasics, patients were more likely to confuse words which began with the same sounds than words which ended with the same sounds. Patients with adequate comprehension at the start of the testing displayed a uniform tendency to improve on the conditions administered later, while those with impaired comprehension at the start usually did not.

Aphasia↗