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Brain SPECT imaging in progressive aphasia.

Progressive aphasia without dementia is a relatively uncommon neurobehavioral syndrome. The initial presentation of progressive aphasia without cognitive impairment may pose a diagnostic dilemma. The authors report a patient with progressive aphasia. The initial neuro-imaging studies (MRI, CT) were not diagnostically helpful. However, brain SPECT imaging with Tc-99m HMPAO clearly demonstrated a left temporal lobe abnormality. This report discusses the potential clinical usefulness of brain SPECT imaging in the evaluation of neurobehavioral disorders.

Aphasia↗

Semantic dementia and primary progressive aphasia: a problem of categorization?

The relationship between semantic dementia (SD) and primary progressive aphasia (PPA) has been the subject of debate ever since the syndromes were first described, in converging streams of research from the neuropsychological and neurologic communities. The most salient clinical features of SD are anomia with circumlocution and semantic paraphasia, single-word comprehension deficit, and reduced category fluency. Of critical importance is the fact that patients also show deficits on non-verbal tasks using visual, auditory, and other modalities, suggesting that the key impairment in SD is a breakdown in conceptual knowledge rather than a specific problem with language. The finding of item consistency between the various tests supports this view. The order in which the features appear can be explained by the variable degree of redundancy in access to semantic knowledge from the different perceptual modalities. Atrophy is seen in the anterior and inferior temporal lobe rather than in classic language areas, further distancing SD from aphasic syndromes. Semantic dementia and progressive non-fluent aphasia (PNFA) share some clinical and pathologic characteristics with frontal variant frontotemporal dementia, but there are also clear differences between the three syndromes. We believe that many patients described as having fluent primary progressive aphasia in fact have early SD. Semantic dementia is a well-defined syndrome, distinct from PNFA but related to it within the spectrum of frontotemporal lobar degeneration syndromes.

Aphasia, Primary Progressive↗

Optic aphasia: a process of interaction between vision and language.

A neurological syndrome, called in the literature ether optic aphasia or visual anomia, is defined in principle as the inability to name visually presented objects, together with the preservation of both the ability to identify them by sight correctly and to name them when they are presented in another sensory modality. This syndrome was first described by Freund in 1889, but since then its existence has been continually questioned. When it is accepted, the most common interpretation of it is in terms of an anatomical visuo-verbal disconnection. However, the precise level of the psychological process impaired remains unspecified. The purpose of this paper is threefold. First, evidence is reported showing that a verbal impairment specific to visually presented objects can be observed, as well as analogous syndromes (e.g. tactile aphasia). Secondly, a particular kind of visuo-verbal impairment is defined and called optic aphasia, to distinguish it from other possible cases of visuo-verbal impairments. This syndrome is defined by the specification of the level of the particular psychological process supposed to be impaired, i.e. a disturbance between visual semantics and verbal semantics, both of which operate normally. Thirdly, three hypotheses concerning the operation of the semantic system in normal subjects are derived from the evidence coming from this syndrome.

Anomia↗

Corticobasal degeneration as cause of progressive non-fluent aphasia: clinical, radiological and pathological study of an autopsy case.

A Japanese male developed gradual loss of spontaneous speech at age 60. Three years later meaningful speech had deteriorated to the point that it had become restricted to monotonous utterances. Neuropsychological examination at age 62 showed that he had severe non-fluent aphasia. A brain MRI demonstrated mild cortical atrophy with ischemic lesions in the cerebral white matter. He was diagnosed as having primary progressive aphasia. At age 63, he was admitted to the hospital to reevaluate the neurological condition. Neurologic examination showed severe non-fluent aphasia, hyperreflexia, snout and sucking reflexes. No alien hand was observed. He was able to walk, dress, wash himself and use chopsticks as well as name real objects. At age 65, 99Tc-hexamethylpropyleneamine oxime single photon emission computed tomography (HMPAO-SPECT) revealed diffuse cerebral hypoperfusion that was particularly prominent in the left frontal lobe. An MRI showed progressive cortical atrophy with the definite atrophy of the left paracentral gyrus. The hippocampal formation and putamen were also atrophic. He died of pneumonia at age 67. The brain weighed 810 g with atrophy of the frontal lobe, globus pallidus, enlargement of the lateral ventricles and depigmentation of the substantia nigra. Microscopic examination showed severe neuronal loss and gliosis in the cerebral cortex, globus pallidus interna and substantia nigra. Ballooned neurons were observed in the cerebral cortex. Gallyas-Braak method revealed numerous astrocytic plaques and argentophilic threads in the cerebrum. Clinical diagnosis of corticobasal degeneration sometimes is difficult in individuals with atypical clinical presentations. More exact clinical and radiological criteria may warrant a diagnosis of corticobasal degeneration.

Aphasia, Primary Progressive↗

Dissociation of visual and auditory language comprehension capacity in aphasia.

Visual language comprehension capacity was compared with auditory language comprehension capacity in 26 aphasic subjects. The Token Test was used for both modalities. By the means of the difference in scores on the two tests three groups were separated, that is (1) a group with no essential difference between the two capacities, (2) a group with superior reading capacity and (3) a group with superior auditory capacity. Particular attention was directed to the second group. Seven subjects showed this type of dissociated comprehension. The group was found to be heterogeneous in their linguistic characteristics. The clinical type of aphasia included not only Wernicke's aphasia but also Broca's aphasia. A possible psychological explanation underlying this phenomenon was attempted. Anatomical correlation was also attempted. The hypothesis of sparing the left inferior parietal lobe is proposed as an explanation of why visual comprehension was better preserved than auditory comprehension.

Adult↗

Epileptic aphasia: a consequence of regional hypometabolic encephalopathy?

A series of 25 children, 13 females and 12 males, who had an acquired communication disorder together with epilepsy, but did not fulfil the strict criteria of the Landau-Kleffner syndrome, was studied. All children had a clinical neurological evaluation, speech and language assessment, an awake and sleep EEG, cranial MRI, SPET scan, and audiometry. Clinical seizures were most often polymorphic in type (17 of 25). Atypical absences were the commonest individual seizure type occurring in 15 cases. All patients had an unequivocal epileptiform EEG. Normal sleep phenomena were only observed in 10 cases, enhancement of epileptiform activity in sleep was seen in 16. Cranial MRI was abnormal in six and normal in 19 cases. The SPET scans were abnormal in 22 of 25 children. The language deficits were classified neurologically as receptive aphasia, 24 of 25; expressive aphasia, 20 of 25; nominal aphasia, eight of 25; articulatory dyspraxia, 10 of 25; and auditory agnosia, nine of 25. It is hypothesized that the loss of communication skills is due to an encephalopathy secondary to the persistent epileptic discharge and manifests as a hypometabolic area on the SPET scan.

Adolescent↗

Aphasia as the sole manifestation of simple partial status epilepticus.

Aphasia due to simple partial status epilepticus is rare, particularly in the absence of a seizure history. No previous report describes acute aphasia as the sole clinical manifestation of EEG-monitored status epilepticus, with prompt resolution with treatment. We report a 45-year-old man with a left temporal glioblastoma who acutely developed a global aphasia, during which an EEG revealed continual repetitive sharp waves emanating from the left hemisphere. After injection of i.v. diazepam, the EEG seizure activity ceased, and the patient's language output returned to preseizure levels.

Aphasia↗

Varieties of progressive non-fluent aphasia.

We report four patients with progressive aphasia of the non-fluent type as the presenting clinical manifestation. The patients were included in a longitudinal study of focal progressive neuropsychological syndromes, and were periodically submitted to neuropsychological evaluations and neuroimaging studies (TC, MRI, SPET or PET). The pattern of neuropsychological impairment was in good agreement with the results of functional imaging studies, which indicated involvement of the anterior regions of the left hemisphere. The evolution of the clinical picture was extremely heterogeneous in the four patients, ranging from a relatively stable picture of transcortical motor aphasia to a severe progressive frontal lobe syndrome. Progressive non-fluent aphasia appears to be a reliable clinical marker of the localization of the pathological process; whether this is related to specific neuropathological conditions, such as Pick's disease, remains for the moment a matter of speculation.

Adult↗

A longitudinal study of sentence comprehension difficulty in primary progressive aphasia.

CONTEXT: Patients with primary progressive aphasia have sentence comprehension difficulty, but the longitudinal course of this deficit has not been investigated. OBJECTIVE: To determine how grammatical, single word meaning, and working memory factors contribute to longitudinal decline of sentence comprehension in primary progressive aphasia. We hypothesised partially distinct patterns of sentence comprehension difficulty in subgroups of patients with progressive non-fluent aphasia (PNFA) and semantic dementia (SD). DESIGN: Cohort. SETTING: Institutional out patient referral centre. PATIENTS: PNFA (n = 14), SD (n = 10). MAIN OUTCOME MEASURE: Sentence comprehension accuracy. RESULTS: PNFA patients were significantly impaired at understanding grammatically complex sentences when first seen, and this was more evident than impairment of their comprehension of grammatically simple sentences (p<0.05). Comprehension of grammatically complex sentences correlated with their working memory deficit at presentation (p<0.05). PNFA patients showed modest decline over time in grammatical comprehension. In SD, comprehension of grammatically complex sentences was not more impaired than comprehension of grammatically simple sentences when first seen, but these patients demonstrated a significant longitudinal decline in understanding grammatically complex sentences (p<0.05). Cox regression analyses showed that a deficit in single word meaning contributes to the progressive impairment for grammatically complex sentences in SD (p<0.05), but working memory does not contribute to longitudinal decline in PNFA. CONCLUSION: Patients with PNFA and SD have sentence comprehension difficulty, but distinct factors contribute to this impairment during the course of their disease.

Aged↗

Evaluation and outcome of aphasia in patients with severe closed head trauma.

In this study long-term observation of 12 patients with aphasia secondary to severe closed head trauma took place. The most frequent symptoms were amnestic aphasia and verbal paraphasia. Only one patient with a constant slow wave EEG focus in the dominant hemisphere had severe receptive symptoms. In all other patients the aphasia recovered rather well, though not totally, but the presence and degree of concomitant neuropsychological disorders were most important for the final outcome.

Adult↗

The temporal sequence of aura-sensations in patients with complex focal seizures with particular attention to ictal aphasia.

The sequences of aura sensations in 143 patients with complex partial seizures, were analysed with special emphasis on aphasic symptoms. Anxiety, epigastric sensation and visual hallucination were experienced early in the course of the aura, while illusion of familiarity and aphasia occurred late in the course of the aura. Three groups of interconnections of aura sensations were found which corresponded possibly to the types of seizure constellations proposed by Weiser. Close interconnections between impairment of verbal comprehension during seizures and paroxysmal thought disorder, as well as between paroxysmal paraphasia and illusion of familiarity were noted. Paroxysmal aphasia in patients with complex partial seizures was characterised as a positive symptom in contrast to stable aphasia.

Aphasia↗

Ataxic hemiparesis associated with transcortical motor aphasia.

Two patients, ages 51 and 76 years, suddenly developed transcortical motor aphasia combined with pyramidal signs and ataxia of the right extremities that affected the leg more severely than the arm. Computed tomography revealed nonenhancing lucencies in the distribution territory of the left anterior cerebral artery involving the genu of the corpus callosum, white matter ventral to the left frontal horn, medial primary motor area, supplementary motor area, and superior part of the premotor area. It is postulated that aphasia, ataxia, and weakness resulted from involvement of the supplementary motor area and deep white matter, premotor, and primary motor areas, respectively. Anterior cerebral artery infarctions of the dominant hemisphere may result in transcortical motor aphasia associated with contralateral ataxic hemiparesis.

Aged↗

Aphasia in acute stroke.

Previous surveys of stroke populations have offered only cursory information on language disturbance, and, conversely, few surveys of aphasic populations have dealth exclusively with stroke or with acute phenomena. This paper describes aphasia in 850 acute stroke patients consecutively registered by the Harlem Regional Stroke Program, of whom 177 (21%) were aphasic; of these, nine were of Broca's type, 24 were of Wernicke's type, 14 were of anomic, ten were conduction, seven were of "isolation" type, and 107 were "mixed." An unexpected finding was a significant over-representation of men among the nonfluent aphasics. During the following four to 12 weeks, 12% of fluent aphasics died, and 12% remained moderately or severely impaired; among survivors, aphasia improved in 74%, and in 44% it cleared completely. During the same period, 32% of nonfluent aphasics died, and 34% remained moderately or severely impaired; among survivors, aphasia improved in 52%, and in only 13% did it clear completely. In both fluent and nonfluent groups, hemiparesis and/or visual field cut were associated with poor prognosis.

Acute Disease↗

A double-blind, placebo-controlled study of the use of amphetamine in the treatment of aphasia.

BACKGROUND AND PURPOSE: A number of studies suggest that drugs which increase the release of norepinephrine promote recovery when administered late (days to weeks) after brain injury in animals. A small number of clinical studies have investigated the effects of the noradrenergic agonist dextroamphetamine in patients recovering from motor deficits following stroke. To determine whether these findings extend to communication deficits subsequent to stroke, we administered dextroamphetamine, paired with speech/language therapy, to patients with aphasia. METHODS: In a prospective, double-blind study, 21 aphasic patients with an acute nonhemorrhagic infarction were randomly assigned to receive either 10 mg dextroamphetamine or a placebo. Patients were entered between days 16 and 45 after onset and were treated on a 3-day/4-day schedule for 10 sessions. Thirty minutes after drug/placebo administration, subjects received a 1-hour session of speech/language therapy. The Porch Index of Communicative Ability was used at baseline, at 1 week off the drug, and at 6 months after onset as the dependent language measure. RESULTS: Although there were no differences between the drug and placebo groups before treatment (P=0.807), by 1 week after the 10 drug treatments ended there was a significant difference in gain scores between the groups (P=0.0153), with the greater gain in the dextroamphetamine group. The difference was still significant when corrected for initial aphasia severity and age. At the 6-month follow-up, the difference in gain scores between the groups had increased; however, the difference was not significant (P=0.0482) after correction for multiple comparisons. CONCLUSIONS: Administration of dextroamphetamine paired with 10 1-hour sessions of speech/language therapy facilitated recovery from aphasia in a small group of patients in the subacute period after stroke. Neuromodulation with dextroamphetamine, and perhaps other drugs that increase central nervous system noradrenaline levels, may facilitate recovery when paired with focused behavioral treatment.

Adrenergic Uptake Inhibitors↗

Treatment of acquired epileptic aphasia with the ketogenic diet.

Acquired epileptic aphasia remains a poorly understood entity, which is frequently difficult to treat. Previously described treatment modalities have included antiepileptic drugs, corticosteroids, intravenous immunoglobulin, and multiple subpial transections. We describe three patients with acquired epileptic aphasia refractory to traditional treatments who were successfully treated with the ketogenic diet. All three patients had lasting improvement of their language, behavior, and seizures for 26, 24, and 12 months, respectively. This is the first reported series of patients with acquired epileptic aphasia successfully treated with the ketogenic diet, and suggests a new therapeutic alternative for patients with this often difficult-to-treat disorder.

3-Hydroxybutyric Acid↗

Progressive nonfluent aphasia with dementia: a case report.

We report a patient with progressive nonfluent aphasia and mild dementia. On 9-month follow-up evaluation, there was significant deterioration in both language and cognition. Unlike in Alzheimer's disease, the aphasia was of the Broca's type. In contrast with primary progressive aphasia, a dementia was present and the cognitive and language decline showed a rather rapid course.

Alzheimer Disease↗

Prognostic value of EEG in acute vascular aphasia: a long term clinical-EEG study of 53 patients.

A group of 53 patients rendered acutely aphasic by occlusive cerebrovascular disease were studied by serial EEG's, repeated neurologic examinations and speech evaluations (Porch Index of Communicative Ability) over a period of eight to twenty-four months, in order to correlate EEG findings with the degree of language disorder and prognosis for language recovery. Normal and mildly abnormal EEG's, posterior slow foci, focal slowing of semirhythmic type and higher alpha frequencies over the intact hemisphere correlated with good language recovery. In the majority of the patients, the curves of "EEG Improvement" and "Language Recovery" closely paralleled each other. These data indicate that the EEG is of prognostic value as to recovery from aphasia in patients suffering from acute occlusive cerebrovascular disease. Despite the advent of newer diagnostic tests, such as CAT scan, which has established its value in evaluation of the anatomy of aphasia, (9) EEG remains to be useful as a tool that could predict the outcome of aphasia in stroke patients.

Adult↗

Crossed aphasia in multiple sclerosis.

Aphasia is a rare sign of multiple sclerosis (MS). Several different forms of aphasia have been reported in MS. We report, to our knowledge, the first case of a MS patient with crossed aphasia during an attack.

Adult↗