Focal pachymeningitis in a returning traveler: Don't forget melioidosis.

Background: Melioidosis is an endemic disease in South-East Asia and Northern Australia caused by a Gram-negative bacillus, Burkholderia pseudomallei. Manifestations are wide and neurological involvement have rarely been described. Methods: In this paper, we report a patient returning from Asia with an unusual infection including CNS involvement consistent with a melioidosis. Results: This diagnosis was challenging and complex to carry out with multiple considerations, mainly because of the atypical nature of the germ. Burkholderia pseudomallei can be easily misidentified with Burkholderia thailandensis (rarely pathogenic to humans) during bacterial culture because of their phylogenetic proximity. The main pitfall of the management was that the responsible infectious agent was not referenced in the MALDI-TOF (considered as a bioterrorism agent) and led to a wrong strategy. Conclusions: This case of melioidosis shows the difficulty regarding the diagnosis of this disease in a patient returning from an endemic zone and its frequent multiple organs involvement. Melioidosis is an emerging, potentially fatal disease which requires prolonged antibiotic treatment. Difficulties in clinical microbiology laboratories diagnosis of melioidosis, especially in non-endemic areas where clinical suspicion is low, may delay treatment and affect disease outcomes.

Pathological laughing and psychotic disorder: the medical evaluation of the Joker.

In the psychological thriller film Joker, released in 2019 and starring Joaquin Phoenix in the first role, another possible origin story for this iconic character is reported. Above all, it brings us medical elements for the understanding of the development of this complex character. Contrary to other interpretations, we discover a lonely, timid and uncharismatic man (Arthur Fleck). He seems to be suffering from psychobehavioral disorders and seems depressed. There is a strangeness in his behavior along with social withdrawal. He suffers from fits of laughter that occur at socially inappropriate times. He also suffers from psychotic symptoms with visual delusions. We learn through the film that he was a beaten child, psychologically and physically abused with severe traumatic brain injury (TBI). The uncontrollable outbursts of laughter, behavioral and psychotic disorders followed these elements. As a neurologist, I was intrigued by these symptoms. I have explored the neuropsychiatric symptoms complicating TBI from which he seems to suffer and which have been reported in the literature. We can assume that the Joker is suffering from neuropsychiatric sequelae related to childhood TBI involving the frontotemporal regions and, in particular, the lateral aspect of the left frontal lobe. The movie Joker has medical significance and covers social aspects of medicine and health care. First, it allows us to discuss whether psychotic disorder due to TBI should be considered a neurobiological syndrome. More broadly, albeit fictitious, it asks us about the management of patients with neuropsychiatric illness, which is a public health problem. It also reminds us that semiological descriptions of patients with neuropsychiatric disorders have served as inspiration for many authors.

Tardive Reactivation of Progressive Multiple Sclerosis During Treatment with Biotin.

Multiple sclerosis (MS) is a common autoimmune disease of the central nervous system, causing neurological disability in young adults. A growing understanding of its immunopathogenesis has led to an expanding array of therapies. Notable new advances in disease-modifying therapies for relapsing forms of multiple sclerosis that are based on anti-inflammatory activity have recently been developed. Management of progressive MS is still challenging. Data published in 2014 suggested that daily high doses of biotin, a vitamin involved in myelin synthesis, might have a beneficial impact on disability and progression in progressive MS. However, some patients worsened while on biotin without any clear explanation for this effect. We report the case of a 41-year-old patient suffering from primary progressive (PP) MS who presented after 16 months of treatment with high doses of biotin (QIZENDAY) with worsening of his Expanding Disability Status Scale (EDSS) score and the appearance of a symptomatic new T2 pseudo-tumoural lesion on brain magnetic resonance imaging (MRI), suggestive of tardive inflammatory reactivation possibly due to the biotin. The newer and more effective therapies for MS are, however, associated with risks that necessitate an active management strategy and continuous vigilance. Physicians should be aware of iatrogenic neurological complications and the possible paradoxical effects of biotin. Future treatment approaches to progressive MS must include identification of a biomarker of disease activity. The study of neurofilaments in the cerebrospinal fluid (CSF) and the serum could be of interest when determining the optimal treatment strategy.

JC Virus Granule Cell Neuronopathy and Lymphoma.

Neurological opportunistic infections are going to increase. Clinicians should be aware of the neurological spectrum of JC virus manifestations, including granule cell neuronopathy. Detection of JC virus DNA by polymerase chain reaction in cerebrospinal fluid should be realized in the assessment of a progressive cerebellar ataxia in an immunocompromised patient.

Further evidence for a distinctive atypical degenerative parkinsonism in the Caribbean: A new cluster in the French West Indian Island of Martinique.

BACKGROUND: A high prevalence of an atypical levodopa-resistant parkinsonism has been reported in the Caribbean island of Guadeloupe. These seminal observations have not been replicated or extended to neighbouring populations who share genetic and environmental characteristics. METHODS: To further characterise this atypical parkinsonism we prospectively investigated 305 consecutive patients with neurodegenerative parkinsonism in a community-based population from Guadeloupe and Martinique, a neighbouring French Caribbean island where the population has similar environmental and genetic backgrounds. The aims of this study were to confirm the frequency of atypical parkinsonism within this cohort and to precisely define its clinical phenotype. RESULTS: A high frequency (66%) of atypical parkinsonism was identified in both Guadeloupe and Martinique. The clinical phenotype consisted of a levodopa-resistant parkinsonism with postural instability (72%), early dementia (58%), dysautonomia (58%), rapid-eye-movement sleep behavioural disorder (53%), hallucinations (43%), and supranuclear gaze palsy (29%). A low educational level was identified as a major risk factor for developing atypical parkinsonism (p < .001). CONCLUSION: Our findings support the existence of a distinctive atypical parkinsonism - Caribbean Parkinsonism - within the French Caribbean Islands. This could either correspond to a single entity or reflect a propensity for developing more widespread and rapidly progressive lesions in Caribbean patients with parkinsonism. In both cases, genetic susceptibility and/or environmental exposure may be involved.