Biomedical subjects
Publications and source records attributed to M L Berthier.
Three patients with hemiballismus received intravenous flumazenil and placebo in a double-blind cross-over study aimed to investigate whether an antagonistic action at the level of the GABA-benzodiazepine receptors may improve choreoballistic movements. No clinical benefit was seen.
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OBJECTIVE: The purpose of the study was to examine behavioral/cognitive and neuroradiological features of patients with concurrent Tourette's disorder (TD) and Asperger's syndrome (AS). METHODS: The authors studied the occurrence of structural brain abnormalities using magnetic resonance imaging (MRI) in seven males with concurrent TD and AS, and in nine age-matched males, who had TD but did not have AS. Both groups were tested with an extensive battery of neurological and psychiatric rating scales and cognitive tests. RESULTS: Five of the seven patients with TD and AS had developmental brain anomalies. In contrast, normal MRI scans were found in all but one TD patient without AS. Both groups were not significantly different in the severity of motor and phonic tics, obsessionality, depression and anxiety, or in measures of general intelligence, memory, and language function; but patients with TD and AS had a history of more psychiatric hospitalizations, poor academic achievement, more neurological soft signs and appeared more impaired on complex problem-solving and spatial tests than did TD patients without AS. CONCLUSION: These findings suggest that structural cortical and subcortical abnormalities are more common among individuals with concurrent TD and AS than among sex- and age-matched TD patients without AS. Dysfunction of frontal-subcortical systems may play a role in the pathophysiology of concurrent TD and AS.
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An 81-year-old woman had the sudden onset of left-sided ballismus and an accompanying behavioral change characterized by elation, distractibility, inflated self-esteem, and insomnia, suggesting secondary mania. An MRI revealed a small ischemic infarction of the right thalamus.
Two individuals with Asperger's syndrome, a rare pervasive developmental disorder, developed recurrent episodes of hypersomnia and abnormal behavior (Kleine-Levin syndrome) during adolescence. The possible etiological role of developmental structural brain anomalies and the differential diagnosis of recurrent hypersomnia and abnormal behavior in patients with pervasive developmental disorders are discussed.
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Although mania is a rare complication of brain lesions, recent reports have emphasized the importance of lesion location and genetic predisposition in these patients. In the present study we compared patients who developed a bipolar affective disorder (i.e., mania and depression) after a brain lesion with patients who only developed mania. Although no significant between-group differences were found on demographic variables, the manic-depressed group showed significantly more impairments on the Mini Mental State Exam than the mania only group. All the bipolar patients had subcortical lesions (mainly right head of the caudate and right thalamus), while patients with unipolar mania had significantly higher frequency of cortical involvement (mainly right orbitofrontal and basotemporal cortices). It is suggested that subcortical and cortical right hemisphere lesions may produce different neurochemical and/or remote metabolic brain changes that may underlie the production of either a bipolar disease or a unipolar mania.
While a relative preservation of repetition in acute transcortical aphasia (TA) has usually been associated with the functional integrity of the speech dominant (left) perisylvian area, recent amytal data (Bando et al., 1986) have suggested a fundamental role of the nondominant (right) hemisphere in language repetition. The neuroradiological correlates of repetition were studied in a consecutive series of 21 patients with acute TA. A similar frequency of either perisylvian or extraperisylvian pathology was found. In 2 patients with perisylvian pathology, the injection of amytal in the hemisphere contralateral to the lesion abolished repetition. Positron emission tomography (PET) in another patient revealed marked hypometabolism over the entire left cortical mantle ipsilateral to a basal ganglia lesion, suggesting that preserved repetition was carried out by right hemisphere structures. This was confirmed in a second patient with left extraperisylvian pathology, in whom a second lesion in the right hemisphere resulted in impaired repetition. These findings suggest that the spared contralateral hemisphere may subserve residual repetition in some transcortical aphasic patients with a lesion within or outside the speech-dominant perisylvian area.
Posterior cortical atrophy (PCA) is characterised by slowly progressive dementia with cognitive and perceptual deficits suggestive of bilateral parieto-occipital disease. A case is reported of a patient with PCA and neuropathological findings consistent with Alzheimer's disease.
The pattern of brain asymmetries was visualized on computed tomography (CT) scan in patients with a single acute cerebrovascular lesion. Patients were divided into those with typical or reversed frontal and/or occipital asymmetries. Among patients with a typical occipital asymmetry, those with left frontal or left basal ganglia lesions showed a significantly higher frequency of major depression and significantly higher depression scores than patients with similar lesion location but with reversed occipital asymmetry or those with a typical asymmetry and lesions in other (left or right) brain areas. Among patients with a reversed occipital asymmetry, there was no significant association between left frontal or left basal ganglia lesions and depression. This study demonstrates that the previously reported significant association between post-stroke major depression and lesion location is restricted to patients with a typical occipital asymmetry and is not present in patients with a reversed occipital asymmetry.
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We present a consecutive series of 8 patients who developed a manic episode after a brain injury. Five patients had cortical lesions (4 with damage to the right basotemporal region, and 1 with bilateral damage to the orbitofrontal area). While the other 3 patients had subcortical lesions (white matter of the right frontal lobe, right anterior limb of the internal capsule, and right head of the caudate), a fluorodeoxyglucose positron emission tomography scan showed hypometabolism in the right lateral basotemporal region in all 3 patients. These findings suggest a major role for the basal region of the right temporal lobe in the modulation of mood.
We performed a selective Wada test on a bilingual patient. While a left middle cerebral artery (MCA) injection produced global aphasia for both (Spanish and English) languages, the patient could only speak Spanish (his mother tongue) 1 min after he started to speak English. No language disturbances were observed after a right MCA amytal injection. These findings suggest that all of a multilingual's languages are stored within the verbal-dominant perisylvian region, and while the second language may be organized within the central Sylvian core, the first language may be better represented in more distal perisylvian areas.
Magnetic resonance imaging (MRI) scans were performed on 13 high-functioning male autistic subjects and 13 male nonautistic control subjects comparable in age and nonverbal IQ. Scans were rated for the presence of cerebral cortical malformations. Five autistic subjects had polymicrogyria, one had schizencephaly and macrogyria, and one had macrogyria. None of the control subjects had abnormalities of this type. These abnormalities result from a defect in the migration of neurons to the cerebral cortex during the first 6 months of gestation. The detection of these malformations by MRI, their pathogenesis, and the implications regarding the pathogenesis of autism are discussed.
Recent neuropathological and magnetic resonance imaging (MRI) studies have demonstrated developmental disturbances of neuronal migration in the cerebral cortex of autistic subjects. We report neuroradiological findings in two patients with Asperger's syndrome, a less severe variant of autism. One patient had left frontal macrogyria; the other had bilateral opercular polymicrogyria. A computer tomography (CT) scan performed on one of the patients' first-degree relatives (who had a bipolar disease) also showed cortical migration anomalies.