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Several postmortem studies have shown iron accumulation in the substantia nigra of Parkinson's disease patients. Iron concentration can be estimated via MRI-R 2 ∗ mapping. To assess the changes in R 2 ∗ occurring in Parkinson's disease patients compared to controls, a multicentre transversal study was carried out on a large cohort of Parkinson's disease patients (n = 163) with matched controls (n = 82). In this study, 44 patients and 11 controls were removed due to motion artefacts, 21 patient and 6 controls to preserve matching. Thus, 98 patients and 65 age and sex-matched healthy subjects were selected with enough image quality. The study was conducted on patients with early to late stage Parkinson's disease. The images were acquired at 3Tesla in 12 clinical centres. R 2 ∗ values were measured in subcortical regions of interest (substantia nigra, red nucleus, striatum, globus pallidus externus and globus pallidus internus) contralateral (dominant side) and ipsilateral (non dominant side) to the most clinically affected hemibody. As the observed inter-subject R 2 ∗ variability was significantly higher than the disease effect, an original strategy (intrasubject subcortical quantitative referencing, ISQR) was developed using the measurement of R 2 ∗ in the red nucleus as an intra-subject reference. R 2 ∗ values significantly increased in Parkinson's disease patients when compared with controls; in the substantia nigra (SN) in the dominant side (D) and in the non dominant side (ND), respectively (P SN&#95;D and P SN&#95;ND  < 0.0001). After stratification into four subgroups according to the disease duration, no significant R 2 ∗ difference was found in all regions of interest when comparing Parkinson's disease subgroups. By applying our ISQR strategy, R 2(ISQR) ∗ values significantly increased in the substantia nigra (P SN&#95;D and P SN&#95;ND  < 0.0001) when comparing all Parkinson's disease patients to controls. R 2(ISQR) ∗ values in the substantia nigra significantly increased with the disease duration (P SN&#95;D  = 0.01; P SN&#95;ND  = 0.03) as well as the severity of the disease (Hoehn & Yahr scale <2 and ≥ 2, P SN&#95;D  = 0.02). Additionally, correlations between R 2(ISQR) ∗ and clinical features, mainly related to the severity of the disease, were found. Our results support the use of ISQR to reduce variations not directly related to Parkinson's disease, supporting the concept that ISQR strategy is useful for the evaluation of Parkinson's disease., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022. Published by Elsevier Inc.)

Introduction: Approximately 1.5% of dementia is due to curable aetiology. We report an isolated dementia syndrome due to a meningeal relapse of acute promyelocytic leukaemia with favourable outcome after appropriate treatment., Case Report: A 72-year-old woman, in remission of an acute promyelocytic leukaemia, presented a loss of autonomy for several months due to corticosubcortical dementia. Lumbar puncture showed blast cells indicating meningeal relapse of leukaemia. Intrathecal chemotherapy and arsenic trioxide obtained biological and molecular remission as well as restoration of normal cognitive functions., Conclusion: In patients with hematologic past history such as acute promyelocytic leukaemia, an isolated cognitive impairment should alert physicians to search for an isolated neuromeningeal relapse., (Copyright © 2016 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

Unlabelled: Impairment of the Self has been described in frontal-temporal dementia but little research has been carried out in patients with Alzheimer's disease (AD)., Objective: The aim of this study was to explore changes in the self in patients with AD., Method: Forty-seven patients with mild to moderate AD were examined using a semi-structured scale designed to assess the self-concept along three dimensions, namely, the Material Self, the Social Self and the Spiritual Self., Results: The majority of patients (43 out of 47) presented impairment of at least one dimension of the Self. When only one dimension was affected, it was always the Social Self. The severity of impairment of the Self was correlated to the impairment of the semantic autobiographical memory and apathy., Conclusion: The Self is impaired in AD and the Social Self dimension appears to be more vulnerable in AD than other dimensions., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Background: Environmental reduplication which is characterized by reduplication of places has been reported in right hemispheric lesions, particularly but not only in the right frontal region. However, spatial delirium may follow right sub-cortical lesions., Methods: We describe a 53 years-old man who had a reduplicative paramnesia for event alone after an intracerebral haematoma of the right caudate nucleus., Results: MRI Scan showed also an extension of the right caudate nucleus haemorrhage into the ventricular system. Regional cerebral blood flow studied with 99Tcm-HMPAO showed a decrease of perfusion in the right dorso-lateral frontal cortex. To our knowledge, we reported the first case of reduplicative paramnesia of event associated with a right caudate nucleus injury. Similar right frontal deactivation was observed in two cases of reduplicative paramnesia for place, one of them after an infarction of the retro-lenticular portion of the right internal capsulae, the other after a right thalamo-capsular haemorrhage., Conclusion: We suggest that reduplicative paramnesia for event, like the previous cases reported of reduplicative paramnesia for place, may be linked to a subcortical lesion of the frontal lobe inducing a right functional frontal deactivation.

Background: Myoclonus dystonia syndrome (MDS) is an autosomal dominant movement disorder caused by mutations in the epsilon-sarcoglycan gene (SGCE) on chromosome 7q21., Methods: We have screened for SGCE mutations in index cases from 76 French patients with myoclonic syndromes, including myoclonus dystonia (M-D), essential myoclonus (E-M), primary myoclonic dystonia, generalised dystonia, dystonia with tremor, and benign hereditary chorea. All coding exons of the SGCE gene were analysed. The DYT1 mutation was also tested., Results: Sixteen index cases had SGCE mutations while one case with primary myoclonic dystonia carried the DYT1 mutation. Thirteen different mutations were found: three nonsense mutations, three missense mutations, three splice site mutations, three deletions, and one insertion. Eleven of the SGCE index cases had M-D and five E-M. No SGCE mutations were detected in patients with other phenotypes. The total number of mutation carriers in the families was 38, six of whom were asymptomatic. Penetrance was complete in paternal transmissions and null in maternal transmissions. MDS patients with SGCE mutation had a significantly earlier onset than the non-carriers. None of the patients had severe psychiatric disorders., Conclusion: This large cohort of index patients shows that SGCE mutations are primarily found in patients with M-D and to a lesser extent E-M, but are present in only 30% of these patients combined (M-D and E-M).

Hallucinations (mainly visual), psychosis and excessive daytime sleepiness are potential side-effects of dopaminergic treatment. They may require a reduction or suppression of dopaminergic agonists, and the prescription of atypical neuroleptic agents or vigilance-enhancing drugs. The recent description of narcolepsy-like sleep onset in rapid eye movement sleep periods synchronous with hypnagogic hallucinations in patients with dopaminergic-induced psychosis or excessive daytime sleepiness, suggests that the mesodiencephalic lesions may predispose to the psychic effects of dopaminergic treatment. Disease-related mood disorders, sexual compulsions, gambling or levodopa addiction may also be amplified by the antiparkinsonian treatment. These complications illustrate the neuro-psychic aspect of Parkinson's disease: psychic troubles may result from a subtle balance between the direct effects of drugs, the pre-morbid pathological personality and the cortical and subcortical lesions.

The efficiency of pergolide has been confirmed by the study of a group 29 parkinsonian patients. They were relatively young, and the duration of evolution of their Parkinson's disease was more then ten years, with levodopa-induced dyskinesia and fluctuations. In this group of patients with most serious motor disability, it has been possible to improve dyskinesia and fluctuations with a relatively important dosage of pergolide and without increase of levodopa dosage.

Hallucinations (mainly visual), psychosis and excessive daytime sleepiness are potential side-effects of dopaminergic treatment. They may require a reduction or suppression of dopaminergic agonists, and the prescription of atypical neuroleptic agents or vigilance-enhancing drugs. The recent description of narcolepsy-like sleep onset in rapid eye movement sleep periods synchronous with hypnagogic hallucinations in patients with dopaminergic-induced psychosis or excessive daytime sleepiness, suggests that the mesodiencephalic lesions may predispose to the psychic effects of dopaminergic treatment. Disease-related mood disorders, sexual compulsions, gambling or levodopa addiction may also be amplified by the antiparkinsonian treatment. These complications illustrate the neuro-psychic aspect of Parkinson's disease: psychic troubles may result from a subtle balance between the direct effects of drugs, the pre-morbid pathological personality and the cortical and subcortical lesions.

"Orbitofrontal" and "cingulate" striatofrontal loops and the mesolimbic dopaminergic system that modulates their function have been implicated in motivation and sensitivity to reinforcement in animals. Parkinson's disease (PD) provides a model to assess their implications in humans. The aims of the study were to investigate motivation and sensitivity to reinforcement in non-demented and -depressed PD patients and to evaluate the influence of dopaminergic therapy by comparing patients in "on" (with L-Dopa) and "off" (without L-Dopa) states. Twenty-three PD patients were compared, in both the "on" and "off" states, to 28 controls, using: (1) an Apathy Scale; (2) Stimulus-Reward Learning, Reversal, and Extinction tasks; and (3) a Gambling task. PD patients were found: (1) mildly apathetic; (2) impaired on Stimulus-Reward Learning and Reversal, but not on Extinction; and (3) able to progress in the Gambling task during the first, but not the second assessment. There was no significant correlation between these various deficits. L-Dopa treatment clearly improved motivation, but had more limited and contrasting effects on other variables, decreasing the number of omission errors in Reversal, but increasing the number of perseveration errors in Extinction. These results suggest: (1) an implication of striatofrontal loops in human motivation and explicit and implicit sensitivity to reinforcement; (2) a positive influence of L-Dopa treatment on the subjective evaluation of motivation, but contrasting effects on reward sensitivity.

Levodopa-induced dyskinesias (LIDs) in patients with Parkinson's disease are considered to result from the severity of dopaminergic denervation in the striatum, which is an irrevocable phenomenon, and sensitization induced by long-term intermittent administration of levodopa. Taking advantage of the 64% reduction of levodopa treatment allowed in 12 Parkinson's disease patients by continuous high-frequency stimulation of the subthalamic nucleus, we evaluated the severity of parkinsonian motor disability and LIDs during two levodopa challenges performed before the surgical implantation of the stimulation electrodes and after 8.8 months of continuous bilateral subthalamic nucleus stimulation that was interrupted 2 hours before the levodopa test. Motor disability during the "off" and "on" drug periods was unchanged. The severity of LIDs during the "on" period and dystonia during the "off" period decreased by 54% and 62%, respectively. The reduced severity of LIDs in the absence of subthalamic nucleus stimulation demonstrates that the sensitization phenomenon resulting from long-term intermittent levodopa administration is partially reversible.

Disynaptic Ia reciprocal inhibition acts, at the spinal level, by actively inhibiting antagonist motor neurons and reducing the inhibition of agonist motor neurons. The deactivation of this pathway in Parkinson's disease is still debated. Disynaptic reciprocal inhibition of H reflexes in the forearm flexor muscles was examined in 15 control subjects and 16 treated parkinsonian patients at rest and at the onset of a voluntary wrist flexion. Two patients were reassessed 18 h after withdrawal of antiparkinsonian medication. At rest, the level of Ia reciprocal inhibition between the wrist antagonist muscles was not significantly different between patients and controls. In contrast, clear abnormalities of this inhibition were revealed by voluntary movements in the patients. In normal subjects, at the onset of a wrist flexion, Ia reciprocal inhibition showed a large decrease, and we argue that this decrease is supraspinal in origin. On the less affected sides of the patients the descending modulation was still present but lower than in controls; on the more affected sides this modulation had vanished almost completely. These movement-induced abnormalities of disynaptic Ia reciprocal inhibition were closely associated with Parkinson's disease but were probably not dependent on L-dopa. They could play a role in the disturbances of precise voluntary movements observed in Parkinson's disease.