Heavy metals contaminating the environment of a progressive supranuclear palsy cluster induce tau accumulation and cell death in cultured neurons.

Progressive supranuclear palsy (PSP) is a neurodegenerative disorder characterized by the presence of intracellular aggregates of tau protein and neuronal loss leading to cognitive and motor impairment. Occurrence is mostly sporadic, but rare family clusters have been described. Although the etiopathology of PSP is unknown, mutations in the MAPT/tau gene and exposure to environmental toxins can increase the risk of PSP.

Food toxins and the Caribbean Parkinson plus types.

In the 90's, clinico pathological studies have considerably improved the diagnosis of specific and rare neurodegenerative diseases. After a training in Parkinsons' disease in Paris, the author moved to French West Indies (Guadeloupe) and observed a high incidence of atypical parkinsonism with dementia, unresponsive to levodopa. Similar features were observed in Martinique.

The MAPT gene is differentially methylated in the progressive supranuclear palsy brain.

Background: Progressive supranuclear palsy (PSP) is a rare neurodegenerative disease causing parkinsonian symptoms. Altered DNA methylation of the microtubule-associated protein tau gene correlates with the expression changes in Alzheimer's disease and Parkinson's disease brains. However, few studies examine the sequences beyond the constitutive promoter.

A geographical cluster of progressive supranuclear palsy in northern France.

Objective: To describe a cluster of progressive supranuclear palsy (PSP) in northern France. PSP has not been reported in geographical, temporal, or occupational clusters. A unit of Neurology and Neurogeriatrics opened in 2005 at the Centre Hospitalier de Wattrelos, serving the population of Wattrelos and Leers (combined population 51,551) and parts of neighboring towns.

Prevalence of small cerebral bleeds in patients with progressive supranuclear palsy: a neuropathological study with 7.0-Tesla magnetic resonance imaging correlates.

Introduction: Progressive supranuclear palsy (PSP) is a degenerative disease affecting mainly the brain stem, basal ganglia and cerebellum. Associated cerebrovascular lesions, mainly small cerebral bleeds, are frequently observed in some neurodegenerative diseases such as Alzheimer dementia and rare in others such as frontotemporal lobar degeneration. The present post-mortem study investigates the prevalence and distribution of small cerebral bleeds in PSP brains.

Post-mortem 7.0-tesla magnetic resonance study of cortical microinfarcts in neurodegenerative diseases and vascular dementia with neuropathological correlates.

Background: Until recently cortical microinfarcts (CMIs) were considered as the invisible lesions in clinical-radiological correlation studies that rely on conventional structural magnetic resonance imaging. The present study investigates the presence of CMIs on 7.0-T magnetic resonance imaging (MRI) in post-mortem brains with different neurodegenerative and cerebrovascular diseases.

Iron deposits in post-mortem brains of patients with neurodegenerative and cerebrovascular diseases: a semi-quantitative 7.0 T magnetic resonance imaging study.

Background And Purpose: Accumulation of iron (Fe) is often detected in brains of people suffering from neurodegenerative diseases. However, no studies have compared the Fe load between these disease entities. The present study investigates by T2*-weighted gradient-echo 7.

Superficial siderosis of the central nervous system: a post-mortem 7.0-tesla magnetic resonance imaging study with neuropathological correlates.

Background: Superficial siderosis (SS) is a rare finding on T2*-weighted magnetic resonance imaging (MRI), regarded as a radiological marker of cerebral amyloid angiopathy (CAA). The present study investigates with 7.0-tesla MRI the prevalence of SS and its underlying pathological substrate in a consecutive series of post-mortem brains of elderly patients with various neurodegenerative and cerebrovascular lesions.

Hippocampal microbleed on a post-mortem t(2)∗-weighted gradient-echo 7.0-tesla magnetic resonance imaging?

The present post-mortem study of a brain from an Alzheimer patient showed on a T(2)∗-weighted gradient-echo 7.0-T MRI of a coronal brain section a hyposignal in the hippocampus, suggesting a microbleed. On the corresponding histological examination, only iron deposits around the granular cellular layer and in blood vessel walls of the hippocampus were observed without evidence of a bleeding.

Tauopathies with parkinsonism: clinical spectrum, neuropathologic basis, biological markers, and treatment options.

Tauopathies with parkinsonism represent a spectrum of disease entities unified by the pathologic accumulation of hyperphosphorylated tau protein fragments within the central nervous system. These pathologic characteristics suggest shared pathogenetic pathways and possible molecular targets for disease-modifying therapeutic interventions. Natural history studies, for instance, in progressive supranuclear palsy, frontotemporal dementia with parkinsonism linked to chromosome 17, corticobasal degeneration, and Niemann-Pick disease type C as well as in amyotrophic lateral sclerosis/Parkinson-dementia complex permit clinical characterization of the disease phenotypes and are crucial to the development and validation of biological markers for differential diagnostics and disease monitoring, for example, by use of neuroimaging or proteomic approaches.

Geographic isolates of atypical Parkinsonism and tauopathy in the tropics: possible synergy of neurotoxins.

Over the last 60 years an abnormally high prevalence of atypical Parkinsonism has been reported in 5 different geographic isolates. It was first described on Guam, later in New Guinea and in the Kii peninsula, on Guadeloupe, and in New Caledonia. We investigated the phenotype of atypical Parkinsonism in three of these foci and observed several similarities with dementia in most and amyotrophic lateral sclerosis in some.

[Neuroradiological aspects of Devic's neuromyelitis optica].

Introduction: Neuromyelitis optica (NMO) is a rare inflammatory and demyelinating disorder of the central nervous system, restricted to optical nerves and spinal cord. The main neuroradiological aspects, now summarized into a complete set of diagnosis criteria, are a normal cerebral MRI at onset and longitudinal involvement of the spinal cord concerning more than 3 vertebral segments. The clinical course and frequency of typical lesions remain unknown.

[Preliminary pilot study of cyproterone acetate for the treatment of aggressive behavior associated with severe dementia].

Introduction: Behavioral symptoms are common in dementia, and seem to be more frequent in men than in women. Agitation is frequently responsible for caregiver burn-out and leads to institutionalization. The dramatic increase in the prevalence of Alzheimer's disease and related disorders requires better management of behavior symptoms.

Role of return migration in the emergence of multiple sclerosis in the French West Indies.

The emergence of multiple sclerosis in island societies has been investigated only in a few Caucasian populations living in temperate regions. The effect of human migration on the risk of developing this disease is still an open question because of possible genetic selection. We conducted an epidemiological study of the multiple sclerosis population in the French West Indies (Martinique and Guadeloupe), a population which includes large numbers of West Indians who have returned after emigrating to metropolitan France.

Atypical unclassifiable parkinsonism on Guadeloupe: an environmental toxic hypothesis.

We characterize the clinical features of Parkinson's syndrome on Guadeloupe and describe possible environmental causes. Consecutive patients who were referred to the University Hospital at Pointe a Pitre with parkinsonism from September 1996 to May 2002 were included. All cases were examined in a standardized manner by a neurologist with a special interest in movement disorders and independently by 3 external movement disorders specialists, using standard operational clinical diagnostic criteria.

Atypical parkinsonism on Guadeloupe, comparison with the parkinsonism-dementia complex of Guam, and environmental toxic hypotheses.

Introduction: : On Guadeloupe, atypical parkinsonism is abnormally frequent, and represents 75% of progressive parkinsonism while Parkinson's disease (PD) accounts for only 25%, which is an inversed percentage in comparison with Europe. Herbal tea made with Annonaceae leaves (containing benzyltetrahydroisoquinolines (Be-TIQ), tetrahydroprotoberberines (THPB) and acetogenins (potent mitochondrial complex I inhibitors) are commonly used on Guadeloupe.

Clinical Study: : Of 265 patients studied on Guadeloupe, 66 (25%) had PD, and 199 (75%) had atypical parkinsonism.

Bipallidal haemorrhage after ethylene glycol intoxication.

Acute or subacute bipallidal lesion, an uncommon radiological feature produced by metabolic disorders or poisoning, has never been attributed to ethylene glycol (EG) intoxication. This 50-year-old Afro-Caribbean alcoholic man had unexplained loss of consciousness. Blood tests showed osmolar gap.

Detection and determination of reticuline and N-methylcoculaurine in the Annonaceae family using liquid chromatography-tandem mass spectrometry.

In Guadeloupe, the French West Indies, there is a high incidence of atypical parkinsonism or progressive supranuclear palsy, and all of the investigated patients had taken herbal tea or tropical fruits of the Annonaceae family. Local inhabitants consume the fruits, and also drink tea made from the leaves. In the present study, we used liquid chromatography-tandem mass spectrometry (LC/MS/MS) with multiple reaction monitoring (MRM) to detect low-molecular-weight neurotoxic benzylisoquinoline derivatives in the Annonaceae family.

[Subacute motor neuropathy induced by T3 hyperthyroidism].

Subacute motor neuropathy involving bulbar nerves is an unusual complication of hyperthyroidism. Clinical and neurophysiologic follow-up of such patients has been rarely reported. We describe a 41-year-old Colombian patient who developed respiratory failure associated with motor neuropathy and severe weight loss.

[Peroneal nerve palsy induced by anterior tibialis pyomyositis].

A 16-year-old girl from Guadeloup developed paresis of the flexors of the right foot, associated with edema and acute pain located in the upper anterior tibialis muscle. Electromyography confirmed mononeuritis of the right peroneal nerve, with severe reduction of potential amplitude. Computed tomography of the right leg showed a heterogeneous mass involving the upper segment of the anterior tibialis muscle, close to the location of peroneal nerve.

[Wernicke-Korsakoff syndrome: diagnostic contribution of magnetic resonance imaging].

Data regarding the magnetic resonance imaging (MRI) features in Wernicke-Korsakoff syndrome (WKS) are scarce. WKS usually combines a cerebellar syndrome, oculomotor disorder and confusion. The aim of this study was to determine more precisely the clinical presentation of WKS and the frequency and topography of MRI abnormalities.

Progressive supranuclear palsy and its relation to pacific foci of the parkinsonism-dementia complex and Guadeloupean parkinsonism.

Steele, Richardson and Olszweski in 1964 described a distinctive clinical and pathological entity they called progressive supranuclear palsy (PSP). Now on Guadeloupe in the Carribbean French West Indies, Caparros-Lefebvre is identifying many patients with similar clinical and histological features. Others have a clinical syndrome of atypical parkinsonism that resembles the parkinsonism-dementia complex (PDC) of Guam and the Kii peninsula of Japan (PDC).