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Biomedical subjects

P Marien

Publications and source records attributed to P Marien.

16 recordsLinked to original sources

Anomalous cerebral language organization: acquired crossed aphasia in a dextral child.

Following a dramatic change of its reported incidence, it was only recently recognized that acquired crossed aphasia in dextral children represents a highly exceptional phenomenon. We describe in a three epoch time-frame model the aphasic and neurocognitive manifestations of an additional case and focus briefly on its anatomoclinical configurations. In our patient, a right parietal cortico-subcortical hemorrhagic lesion caused an initially severe aphasia. After remission of the global aphasic symptoms in the acute phase, an adynamic output disorder with relatively severe auditory-verbal comprehension disturbances developed. In addition to the adynamia of self-generated speech, formal language investigations performed 3 weeks postonset, revealed agrammatism, hypertonic dysarthria, and dysprosodia. A substantial improvement of the aphasic disorder was objectified 83 days postonset. Neuropsychological investigations disclosed both dominant and nondominant hemisphere dysfunctions. Reassessment of neurocognitive functions after a 10-year period evidenced discrete residual anomia, confined to visual confrontational naming and a discrete visuo-perceptual syndrome. Given the posterior localization of the lesion, the syndrome shift from global to predominantly adynamic aphasia represents a finding beyond the plausible anatomoclinical expectations holding in general for the uncrossed, classic types of childhood and adult aphasia. As the first representative of crossed aphasia in dextral children with an anomalous lesion-aphasia profile, our case provides evidence to enrich the discussion on lateralization and intrahemispherical organization of language functions in both childhood and adult aphasia.

Acute Disease↗

Acquired crossed aphasia in dextral children revisited.

In contrast to the estimated low incidence of crossed aphasia in dextral adults (among 1%), crossed aphasia in children has been considered a common finding for almost a century. However, reviewing the literature on crossed aphasia in dextrals (CAD) and its related topics from 1975 onward, we encountered only 5 children in a corpus of 180 cases (2.7%). Critical analysis rendered three of the reported cases ambiguous and hence not suitable to draw potentially relevant conclusions. In this review, the neurobehavioral manifestations of the two representative childhood CAD cases are analyzed and compared with adult CAD and acquired childhood aphasia (ACA). In the light of our findings, which support the position of innate cerebral specialization for language, the long-standing controversy as to whether lateralized hemispheric specialization for language is innate or develops progressively during maturation is briefly discussed.

Aphasia↗

The lateralized linguistic cerebellum: a review and a new hypothesis.

During the past 2 decades the collaboration across disciplines and the methodologic and conceptual advances of contemporary neuroscience have brought about a substantial modification of the traditional view of the cerebellum as a mere coordinator of autonomic and somatic motor functions. Growing insights in the neuroanatomy of the cerebellum and its interconnections, evidence from functional neuroimaging and neurophysiological research, and advancements in clinical and experimental neuropsychology have established the view that the cerebellum participates in a much wider range of functions than conventionally accepted. This increase of insight has brought to the fore that the cerebellum modulates cognitive functioning of at least those parts of the brain to which it is reciprocally connected. This article reviews the recently acknowledged role of the cerebellum in cognition and addresses in more detail experimental and clinical data disclosing the modulatory role of the cerebellum in various non-motor language processes such as lexical retrieval, syntax, and language dynamics. In agreement with the findings indicating a topographical organization of the cerebellar structures involved in language pathology we advance the concept of a "lateralized linguistic cerebellum." In our view crossed cerebral diaschisis processes, reflecting a functional depression of supratentorial language areas due to reduced input via cerebellocortical pathways, might represent the relevant pathomechanism for linguistic deficits associated with cerebellar pathology.

Aphasia↗

Phonological agraphia following a focal anterior insulo-opercular infarction.

Following a unique infarction, restricted to the left anterior insula and the adjacent part of the intrasylvian frontal opercular cortex, an 83-year-old right-handed patient acutely developed a severe speech disorder that evolved into mere mutism within a few hours. After rapid recovery from mutism, oral language was characterized by severe apraxia of speech. In-depth language investigations further disclosed an isolated, highly selective disturbance of the spelling system (phonological agraphia) which resolved rapidly. One year after onset of neurological symptoms, the apraxia of speech had almost completely receded. The anatomoclinical findings in this first representative of pure and nearly isolated phonological agraphia complement previous neuroanatomical and neurolinguistic accounts of phonological agraphia. The data not only seem to enrich current insights in the anatomical locus for phonological agraphia, they also seem to contribute to a further delineation of the insular role in phonologically mediated aphasic manifestations.

Aged↗

Cerebellar neurocognition: a new avenue.

The functional role of the cerebellum is traditionally defined as a mere coordinator of automatic and somatic motor functions. This view determined most of the clinical and experimental investigations of the functions of the cerebellum and overshadowed many attempts to expand its role to non-motor functions. However, during the past decades the collaboration across contemporary neuroscience disciplines has brought about a substantial modification of traditional thinking about the cerebellum. Converging evidence from different neuroscientific angles established the view that the human cerebellum participates in a much wider range of functions than conventionally accepted. Studies have brought to the fore that the cerebellum also modulates neurocognitive functions of at least these parts of the brain to which it is reciprocally connected. In less than two decades, the concept of 'cerebellar neurocognition' evolved from a mere afterthought to an exciting new multifaced area of contemporary neuroscientific investigations. Within this area, one of the major avenues of current research is the role of the cerebellum in non-motor language processing. This paper reviews the recently acknowledged role of the cerebellum in neurocognition and linguistic processes.

Animals↗

Loss of psychic self-activation after paramedian bithalamic infarction.

BACKGROUND AND PURPOSE: Loss of psychic self-activation has been described after bilateral lesions to the globus pallidus, striatum, and white matter of the frontal lobes, but it is a very rare sign of bithalamic lesions. The exact functional-anatomic mechanism underlying loss of psychic self-activation following bithalamic lesions remains to be elucidated. CASE DESCRIPTION: We present clinical, neuropsychological, structural, and functional neuroimaging data of an 18-month follow-up period of a man with prominent loss of psychic self-activation after coronary arteriography. Except for memory decline, accompanying symptoms remained restricted to the acute phase. The neurobehavioral syndrome consisted mainly of apathy, indifference, poor motivation, and flattened affect, and this remained unchanged during the entire follow-up period. MRI showed a bithalamic infarction involving the nucleus medialis thalami bilaterally. Single-photon emission CT revealed a severe relative hypoperfusion of both thalami, a relative hypoperfusion of both nuclei caudati, and a relative hypoperfusion mesiofrontally. CONCLUSIONS: Single-photon emission CT data support the hypothesis that the neurobehavioral manifestations after bithalamic paramedian infarction are caused by disruption of the striatal-ventral pallidal-thalamic-frontomesial limbic loop. Probably, bilateral disruption at different levels of the striatal-ventral pallidal-thalamic-frontomesial loop may lead to a similar clinical picture consisting of loss of psychic self-activation.

Affect↗

Gastric emptying in formula-fed and breast-fed infants measured with the 13C-octanoic acid breath test.

BACKGROUND: The 13C-octanoic acid breath test, a noninvasive method for measuring gastric emptying, was used to compare the gastric-emptying rate of formula-fed and breast-fed infants. Octanoic acid, a medium-chain fatty acid marked with the stable isotope 13C is immediately absorbed in the duodenum. Because gastric emptying is the rate-limiting step for the absorption of medium-chain fatty acids, the fraction of 13C expired in the breath indicates the rate of gastric emptying. METHODS: Twenty-nine newborn infants (16 boys, 13 girls) were investigated, with parental consent. The infants had a mean gestational age at birth of 34.5 weeks (range, 27-41 weeks) and a birth weight of 2148 g (range, 960-4100 g). Their mean weight on the day of the test was 2496 g (range, 1998-4140 g), and their mean age was 23 days (range, 7-74 days). Each infant received a test meal after a maximum fasting period of 3 hours. Fourteen infants were fed formula milk (Nutrilon Premium, NV Nutricia, Zoetermeer, The Netherlands) with 13C-octanoic acid and 15 infants received expressed mother's milk mixed with 13C-octanoic acid. After obtaining two basal breath samples and the feeding, breath samples were collected using a nasal prong, every 5 minutes during the first half hour and every 15 minutes during the next 3.5 hours. Analysis of the expired 13C fraction in the breath samples was performed using isotope-ratio mass spectrometry, and the gastric emptying curve and gastric emptying parameters were determined. RESULTS: The mean half-emptying time determined by the 13C-octanoic acid breath test was 65 minutes (range, 27-98 minutes) for the formula fed infants and 47 minutes (range, 16-86 minutes) for the breast-fed infants. The difference between the half-emptying times is significant (t-test, p < 0.05). CONCLUSIONS: The results of the 13C-octanoic acid breath test indicated faster gastric emptying of human milk than formula. Our findings are in accordance with those in earlier studies, using the invasive-dilution technique; noninvasive and detailed ultrasonography, which is not easily used because it is operator dependent and the observation time is short; or cineesophago-gastroscintigraphy, which is less suitable for infants (because of the radiation involved). The 13C-octanoic acid breath test is a safe and noninvasive method for measuring gastric emptying in small infants and allows comparison of various feeding methods.

Breast Feeding↗

Sensitivity and specificity of 99Tcm-HMPAO single-headed SPECT in dementia.

The sensitivity and specificity of 99Tcm-hexamethylpropyleneamine oxime (HMPAO) single-headed single photon emission computed tomography (SPECT) in dementia were evaluated in elderly patients with a mean age of 84 years suffering from dementia (n = 59) and compared to an age- and sex-matched control group with normal cognitive function (n = 14). The demented patients were classified as suffering from primary degenerative dementia (PDD, n = 51) based on DSM-III-R criteria. Moreover, PDD patients were divided into three subgroups of severity of cognitive impairment, according to their Folstein score. Multi-infarct dementia (MID, n = 8) was diagnosed based on clinical features, computed tomographic findings and Hachinski ischaemic score. Images were assessed qualitatively by visual interpretation of shades of colour in cortical regions. A SPECT defect confined to the frontal, parietal, temporal or (parieto) occipital lobe was defined as one lesion. The mean number of SPECT lesions was 1.4 (range 0-4) in the control group, 2.4 (range 0-8) in the PDD group and 2.9 (range 2-5) in the MID group and showed a significant difference (P < 0.02) between groups. To obtain an acceptable level of specificity of 64.3%, a cut-off value of three lesions had to be chosen. In the PDD group, sensitivity was then 25% for the mildly, 43.8% for the moderately and 46.7% for the severely affected PDD subgroup. In the MID group sensitivity was then 75%. Interestingly, in this elderly patient population the location of lesions was homogenously distributed in all groups, including the control group.

Aged↗

Multiple cerebral infarctions in a young patient with secondary thrombocythemia due to iron deficiency anemia.

A 30-year-old woman developed multiple cerebral infarctions. In the absence of other risk factors, thrombocythemia secondary to iron deficiency anemia due to polymenorrhoea was considered to underlie the cerebral infarctions. Platelet count was normalized after iron therapy. The importance of vigorous treatment of iron deficiency anemia in preventing complications of secondary thrombocythemia is emphasized.

Adult↗

Slow progressive bilateral posterior artery infarction presenting as agitated delirium, complicated with Anton's syndrome.

Three patients presented with an acute agitated delirium as the earliest sign of bilateral posterior cerebral artery infarction. All patients showed a unique slow progressive deterioration with a remarkably long interval between the first neuropsychological and subsequent visual and neurological symptoms, ranging from 3 to 30 days. Repeated CT scans demonstrated hypodensities in the posterior artery territory only after a long interval of 9-12 days, in case 3, and between 33 and 48 days in case 2. In the latter case MRI was still negative 33 days after onset. In 2 patients the cortical blindness was complicated with anosognosia for blindness. Clinical condition worsened progressively in all patients, leading to death, probably due to brainstem infarction. In all 3, the combination of clinical and radiological findings indicated a 'top of the basilar' distribution, which could be confirmed in two by autopsy.

Aged↗

Evolution of technetium-99m-HMPAO SPECT and brain mapping in a patient presenting with echolalia and palilalia.

A 78-yr-old woman presented with transient echolalia and palilalia. She had suffered from Parkinson's disease for 2 yr. Routine laboratory examination showed hypotonic hyponatremia, but was otherwise unremarkable. Brain mapping revealed a bifrontal delta focus, more pronounced on the right. Single photon emission computed tomography (SPECT) of the brain with technetium-99m labeled d,l hexamethylpropylene-amine oxime (99mTc-HMPAO), performed during the acute episode showed relative frontoparietal hypoactivity. Brain mapping performed after disappearance of the echolalia and palilalia, which persisted only for 1 day, was normal. By contrast, SPECT findings persisted for more than 3 wk. Features of particular interest in the presented patient are the extensive defects seen on brain SPECT despite the absence of morphologic lesions, the congruent electrophysiologic changes and their temporal relationship with the clinical evolution.

Aged↗

Distal 11q monosomy. The typical 11q monosomy syndrome is due to deletion of subband 11q24.1.

In this paper we describe two new patients with distal 11q monosomy and precisely localize breakpoints using high resolution banding techniques. The findings in these two patients further contribute to the precise localization of the crucial band for 11q monosomy syndrome as being at 11q24.1. A very distal 11q24.2 deletion in the second patient resulted in a completely different phenotype.

Adult↗