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Landau-Kleffner syndrome (acquired epileptic aphasia) in a Nigerian child.

Epilepsy is one of the commonest disorders presenting the neurological clinics in developing countries. There are attendant complications especially in untreated or poorly treated cases. In this article, we report an uncommon complication known as Landau-Kleffner syndrome in a Nigerian girl. The syndrome, typically seen in children consists of an acquired aphasia frequently associated with clinical seizures and paroxysmal electroencephalographic (EEG) accompaniment. There had been no adequate neuropathlogical correlations to explain the symptomatology; thus, various forms of pathogenesis have been ascribed to the syndrome. The clinical seizure is easily controlled by an antiepileptic; but speech recovery is variable, and this is usually poor in cases presenting one or two years after onset.

Anticonvulsants↗

Subdural hematomas and isolated transient aphasia.

Chronic subdural hematoma (CSDH) is particularly common in the older patient. Chronic subdural hematoma can present with atypical, and sometimes unusual, manifestations. It has an insidious onset and can, at times, be very difficult to diagnose. The most frequent presenting symptoms are headache, changes in mental status, and hemiparesis. Chronic subdural hematoma might also present as a transient ischemic attack. Here we report a case of CSDH that presented with intermittent aphasia and no other neurological deficit. Chronic subdural hematoma usually begins with a hemorrhage into the subdural space, which then becomes encapsulated by a membrane with abundant abnormal vessels in the outer portion of the membrane. Chronic subdural hematoma is a distinct and interesting clinical entity with diverse and insidious onset. A high index of suspicions is needed to make the early diagnosis and to start appropriate treatment to avoid morbidity and mortality.

Journal Article↗

Aphasia quality of life: reliability of the Italian version of SAQOL-39.

AIM: In recent times there has been an increasing interest in assessing the quality of life (QOL) in stroke clinical trials. To our knowledge, an Italian tool suitable for this purpose is still missing. So we adapted to Italian language the Stroke and Aphasia Quality of Life Scale-39 (SAQOL-39), that is a British questionnaire recently validated with aphasic subjects. The aim of this paper is to validate our version of SAQOL-39. METHODS: To determine the matching of our version, we requested a native English speaker to translate the Italian version into English. Twelve patients (7 male, 5 female; mean age 66.4 years) were submitted to our test. All subjects were interviewed twice by 2 raters, at 24 h of distance. Neither examinator knew results of the other interview. Statistical analysis was performed by determining Cronbach's a and the intraclass correlation coefficient (ICC). RESULTS: Comparing the original SAQOL-39 to the English translation of our Italian version, we found only a low percentage (10%) of minimal semantic variations. As for test-retest reliability, ICC for global score was 0.898 (ICC(2,39)). ICC range for SAQOL-39 subdomains was 0.816 to 0.969. Cronbach's a for full scale scores was 0.916 (subdomains ranged 0.767 to 0.976). Results seem good as for usefulness, reliability and acceptability of the Italian version of SAQOL-39, like the original version of this test. CONCLUSIONS: Our Italian version of SAQOL-39 seems suitable for clinical use. A multicentric study aiming to compare our data to original English ones is in progress.

Journal Article↗

Short-term memory and sentence comprehension. An investigation of a patient with crossed aphasia.

The relationship between short-term memory impairment and sentence comprehension is explored in a right-handed patient with a focal temporoparietal lesion of the right hemisphere. The general clinical profile, as well as characteristics of the patient's immediate memory for word lists, suggests the occurrence of a 'mirror image' crossed aphasia. Detailed analysis of the patient's ability to repeat and to comprehend sentences, however, indicates some important differences between this case and previously reported patients with short-term memory impairment. It is suggested that these differences, which may be related to an unusual pattern of neuroanatomical organization of cognitive functions, involve symptom dissociations with implications for models of normal language/memory interactions.

Brain↗

[Right hemiplegia and spatial neglect with apraxia and agraphia without aphasia in a right-handed patient].

A 65 year-old right-handed woman was admitted after the sudden onset of a right dense hemiplegia. C.T. showed a large left infarction in the middle cerebral artery territory. There was a slight anosognosia and neglect of the right space without confusion. She had aprosodia but no aphasia. On the other hand, there was a severe apraxia and all the components of Gerstmann's syndrome were present. This suggests an unusual sattering of hemispheric functional dominances.

Aged↗

Auditory perception in patients with CVA without aphasia.

Tests of central auditory function were performed variously on 64 patients with hemiplegia who had suffered a single stroke but with minimal or no aphasia symptoms and with essentially normal hearing. Both right and left hemisphere lesions were represented for comparison of effects on the tests. Reports of central auditory dysfunction generally assume localization in the temporal lobes. Since it was apparent that our tests were revealing positive findings in patients in whom there was no recognized evidence of temporal lobe involvement we deliberately selected for one study 34 patients without clinical signs of such lesions. The tests required selective listening in which patients had to a) perceive and repeat a prime message in the presence of a simultaneously competing meaningful message, either under earphones or in the sound field (loudspeaker), and b) repeat all words in a classic dichotic listening task. The tests reveal different dysfunctions in right vs left hemisphere damage and document that compensatory transfer of function from the usually dominant left side to the fight hemisphere usually occurs only in young children. The dysfunctions appear to be unrelated to temporal lobe lesions.

Adult↗

[Apraxia and autotopoagnosia without aphasia or agraphia with compulsive language activity in right hemispheric lesion].

A 75 year-old woman was admitted with a left hemiplegia resulting from an infarct in the right middle artery's territory. Her manual preference was ambiguous from early childhood. She presented with severe bilateral apraxia, autotopoagnosia, finger agnosia, and left spatial neglect. There was, however, no aphasia nor agraphia. When the patient attempted to perform gestures on order, she compulsively produced oral or written language. In this very unusual case, dominance for gesture and dominance for language were strictly independent, each ensured by one hemisphere. The patient's performances in gestual activities, especially dissociation between automatic and voluntary movements, and compulsive linguistic productions, are discussed in relation to this functional lateralization. We suggest that the propositional nature of the responses required in test conditions could activate either voluntary language in the left cerebral hemisphere, or voluntary gestures in the right. A competition between the two hemispheres could explain the patient's linguistic apraxic or behavior in response to orders. Autotopoagnosia, an uncommon symptom, could interfere with apraxia, but is not directly responsible.

Aged↗

[A case of biopsy-proven sarcoid meningoencephalitis presented with hallucination, nominal aphasia and dementia].

Here we report a 36-year-old male with sarcoid meningoencephalitis who initially presented with hallucination, nominal aphasia and disturbance of consciousness. After the onset, his symptoms fluctuated. He had no meningeal signs, and his cranial nerves were normal except for bilateral papapilledema. Muscle power and tone was normal, and there was no gait disturbance. Deep tendon reflexes were slightly exaggerated in lower extremities, but no pathological reflexes were noted. There was no impairment in extrapyramidal system, cerebellar system and sensory system. The cerebrospinal fluid revealed increased opening pressure, mild lymphocytic pleocytosis, slightly decreased glucose, increased protein to 87mg/dl and increased IgG index to 1.11. Cerebrospinal fluid culture was negative. Marked meningeal thickening and contrast enhancement was shown by CT and MRI. The meningeal biopsy demonstrated characteristic sarcoid nodules and confirmed the diagnosis of neurosarcoidosis. His manifestations were alleviated by oral administration of corticosteroid. Since neurosarcoidosis is a disorder treatable with corticosteroid, it is important to consider the possibility of this disorder in mind when a patient is presenting with psychiatric manifestations and chronic meningitis.

Adult↗

Perceptual discrimination of vowels in aphasia.

In the present study 3 hypotheses were investigated: first, the notion that an aphasic impairment of vowel perception is not associated with particular aphasic syndromes or lesion sites, second, that it is a disorder comparable to a general impairment of perception in a normal speaker caused by some form of interference, and third, that perceptual phonemic discrimination is a separate process from the phonemic discriminative function necessary for speech production. The hypotheses were tested by means of a vowel discrimination test administered to 50 German-speaking aphasic patients (roughly equally divided between Broca's, mixed non-fluent, Wernicke's and mixed fluent groups); the same test, masked by white noise at -10 dB was also administered to 20 normal native speakers of German. Results were in support of all 3 hypotheses. First, aphasic patients' error patterns were similar across fluent and nonfluent groups and for all lesion sites. Second, the error distributions of aphasics with slight auditory impairment resembled those of normal subjects in the -10 dB white noise condition, while distributions of aphasics with severe auditory impairment were indicative of an added component of guessing behaviour. And third, the patients' performance on the discrimination task differed from that shown on a comparable repetition test. (It was argued that repetition involves a patient's expressive capacity in addition to his perceptual capacity). The differentiation of perceptual and expressive phonemic discrimination was further supported by an analysis of the speech errors occurring in the spontaneous (purely expressive) speech and in the repetition (expressive plus perceptual) tasks of 16 French Canadian and 5 English Canadian aphasics.

Adult↗