P. Rondot

Botulinum antibodies in dystonic patients treated with type A botulinum toxin : frequency and significance

We measured serum antibodies to botulinum toxin (ABT) in 96 patients with focal dystonia who had been treated with type A botulinum toxin. The frequency of detectable ABT was 3% (three patients). Patients with ABT had received more than 50 ng of botulinum toxin, and the shortest time between two injections was significantly less than in patients without ABT. The clinical evolution of the three patients was heterogeneous: one had decreased effectiveness with repeated injections, another had persistent improvement, and the third never responded to toxin injections.

Peduncular 'Rubral' Tremor and Dopaminergic Denervation A PET Study

Article abstract-Lesions causing so-called rubral tremors frequently involve the substantia nigra or the nigrostriatal fibers, suggesting dopaminergic denervation as possibly contributory. We examined this hypothesis using PET and (Fluorine-18)-fluorodopa in six patients with a contralateral tremor following a peduncular lesion. The denervation revealed by PET was even more marked than in severe parkinsonian patients. All patients showed partial to complete improvement with levodopa therapy. PET evaluation of D2-receptors with (Bromine-76)bromolisuride showed no asymmetry of the D2 binding despite the important asymmetry of Fluorine-18-fluorodopa uptake. Our results indicate an important involvement of the nigral dopaminergic system in peduncular tremors that appears to be independent of postsynaptic dopamine receptors. NEUROLOGY 1995;45: 472-477

(99mTc)-HM-PAO SPECT and cognitive impairment in Parkinson's disease: a comparison with dementia of the Alzheimer type.

Regional cerebral perfusion was evaluated by single photon emission tomography (SPECT) using (99mTc)-HM-PAO as a tracer, in thirty Parkinsonian patients with (n = 15) or without (n = 15) dementia, nineteen patients with dementia of the Alzheimer type (DAT) and thirteen control subjects. HM-PAO uptake was measured in the frontal, parietal, temporal and occipital cortex and tracer perfusion was expressed as cortical/cerebellar activity ratios. Regional HM-PAO ratios in nondemented Parkinsonian patients did not differ from controls, whereas in demented patients with Parkinsons disease (DPD) a significant reduction was found in the parietal, temporal and occipital cortex. Tracer uptake ratios were significantly reduced in all regions in the DAT group. Thus DPD and DAT shared a common pattern of marked posterior hypoperfusion, although the perfusion defect was greater and more extensive in the DAT patients.

A comparative technetium 99m hexamethylpropylene amine oxime SPET study in different types of dementia

Regional cerebral perfusion was evaluated by single photon emission computed tomography (SPET) using technetium 99m hexamethylpropylene amine oxime (99mTc-HMPAO) as a tracer, in 13 control subjects and 44 age-matched patients suffering from dementia of the Alzheimers type (DAT, n=19), presumed Picks disease (n=5), idiopathic Parkinsons disease with dementia (DPD, n=15) and progressive supranuclear palsy (PSP, n=5). HMPAO uptake was measured in the superior frontal, inferior frontal, parietal, temporal and occipital cortices, and the perfusion values were expressed as cortical/cerebellar activity ratios. As compared with controls, tracer uptake ratios in the DAT group were significantly reduced over all cortical regions, with the largest defects in the parieto-temporal and superior frontal cortices. A marked hypoperfusion affecting the superior and inferior frontal cortices was found in Picks disease, whereas a mild but significant hypoperfusion was observed only in the superior frontal cortex of patients with PSP. In the DPD group, HMPAO uptake was significantly reduced in the parietal, temporal and occipital cortices, but not in the frontal cortex. These results show that DAT and DPD share an opposite anteroposterior HMPAO uptake defect as compared with the Picks and PSP groups.

Intravascular lymphomatosis (neoplastic angioendotheliosis) of the central nervous system: case report and literature review

SummaryA 63-year-old woman developed gradual slowness, recurrent multifocal deficits, severe constitutional symptoms and hypopituitarism which progressed to death over 2.5 months. Elevation in lactico dehydrogenases was the main biological abnormality. Necropsy showed an intravascular malignant proliferation which proved to be a lymphoma of B-lineage. In order to better define diagnostic criteria, we reviewed previously reported cases of intravascular lymphomatosis of the central nervous system. A strategy for establishing the diagnosis is proposed.

Menzel's hereditary ataxia with slow eye movements and myoclonus: A clinico-pathological study

The patient we describe had cerebellar ataxia, slow eye movements, myoclonus, facial dystonia and signs of spinal cord and peripheral nerve involvement. The patients mother, brother and sister died from the same disease. Neuropathological examination revealed lesions of olivo-ponto-cerebellar atrophy (OPCA) associated with spinal cord degenerative changes characteristic of Menzels hereditary ataxia. Although myoclonus was similar to Hunts dyssynergia cerebellaris myonica, pathological findings did not show significant involvement of the dentate nucleus or superior cerebellar peduncle and physiopathological hypotheses for myoclonus are discussed. Slow eye movement is emphasized in the propositus and we suggest that it could be specific of one type of OPCA. Its pathological significance is discussed, but a primitive and unique involvement of the paramedian pontine reticular formation is unlikely.

EEG Mapping in Pathological Aging and Dementia: Utility for Diagnosis and Therapeutic Evaluation

Quantified EEG (Remond 1961; Drohocki 1968; Walter and Brazier 1968; Dolce and Kunkel 1975; Etevenon 1977) and mapping (Lehmann 1971; Ragot and Remond 1978; Duffy et al. 1981; Buehsbaum etal. 1982) have allowed more precise and reliable EEG analysis than visual methods. However, while the place of these methods in daily clinical practice is being debated they are giving rise to an excess of pictorial and numerical data. Much work has been published concerning aging and dementia (Duffy et al. 1984 a, b; Coben et al. 1985; Penttila etal. 1985; Gueguen etal. 1986; Senant etal. 1986), but only a few studies in the past 2 years have really used EEG as a tool for diagnosis or for therapeutic evaluation (Cutler et al. 1985; Little et al. 1985; Bruno et al. 1986; Delwaie et al. 1986; Partanen et al. 1986; Schwartz and Kolstaedt 1986). EEG changes occur early in dementia and are well correlated with the stage of cognitive impairment (Coben etal. 1985; Penttila etal. 1985). We report here preliminary results concerning the usefulness of quantified EEG methods in diagnosis and treatment of pathological aging.

Ageing and Parkinson’s Disease

Based on the examination of 400 patients suffering from Parkinsons disease, the main symptoms of motor and brain ageing have been underlined. Motor ageing is evidenced by the importance of akinesis, while rigor and quaking are limited, and by the arrival of dystonic attitudes which tend to last. Brain ageing is revealed by memory disturbances, mental disorders and delusions without phasic or praxis troubles. The comparative study of different types of dementia did not allow clinical or anatomopathological confirmation.