Real-World Effectiveness of Natalizumab Extended Interval Dosing in a French Cohort.

Introduction: Natalizumab, a therapy for relapsing-remitting multiple sclerosis (RRMS), is associated with a risk of progressive multifocal leukoencephalopathy (PML). Over the last several years, practitioners have used off-label extended interval dosing (EID) of natalizumab to reduce PML risk, despite the absence of a large-scale efficacy evaluation.

Methods: We conducted a retrospective, multicenter cohort study among adults with RRMS receiving stable standard interval dosing (SID), defined as a ≥ 12-month consecutive period of ≥ 11 natalizumab infusions/year in France.

Narcolepsy secondary to anti-Ma2 encephalitis: two case reports.

Unlabelled: Recent studies suggest that sleep disorders are present in two-thirds of patients with autoimmune encephalitis. In anti-Ma2 encephalitis, hypersomnia appears to be frequent. However, only few cases of type 1 narcolepsy have been reported to date with anti-Ma2 encephalitis.

Safety and efficacy of subcutaneous night-time only apomorphine infusion to treat insomnia in patients with Parkinson's disease (APOMORPHEE): a multicentre, randomised, controlled, double-blind crossover study.

Background: Insomnia is a frequent complaint of patients with Parkinson's disease, and it negatively affects quality of life. Drugs that improve both sleep and parkinsonism would be of major benefit to patients with Parkinson's disease-related insomnia. We aimed to test the safety and efficacy of subcutaneous night-time only apomorphine infusion in patients with Parkinson's disease and insomnia.

Autoimmune cerebellar hypermetabolism: Report of three cases and literature overview.

We report three cases of vermian cerebellar hypermetabolism in patients with autoimmune encephalitis. One of our patients was positive for anti-Ma2 antibodies and one for anti-Zic4 antibodies while the remaining patient did not present any known antibodies. The seronegative patient deteriorated after immune checkpoint inhibitor treatment for a pulmonary adenocarcinoma and improved with immunosuppressive drugs, which is in favour of an underlying autoimmune mechanism.

Phase I trial of bortezomib daily dose: safety, pharmacokinetic profile, biological effects and early clinical evaluation in patients with advanced solid tumors.

Purpose This phase I study investigated bortezomib in solid tumors used as a daily subcutaneous regimen. Previous regimens showed only modest activity in solid tumors which was potentially related to sub-optimal tumor penetration. We aimed at exploring if daily low dose administration of bortezomib may allow a greater and tolerable pharmacokinetic exposure which might be required for antitumor activity in solid tumors.

Headache changes prior to aneurysmal rupture: A symptom of unruptured aneurysm?

Background And Objectives: The symptomatic status of unruptured aneurysms has to be looked for. The objective of this retrospective case-control study was to identify the headache semiologic characteristics of symptomatic aneurysms during the 3 months prior to patient admission.

Patients And Methods: The case cohort was composed of 40 consecutive patients admitted for the treatment of a ruptured intracranial aneurysm (IA) and able to answer a standardized questionnaire by the same neurologist.

Quick, non-invasive and quantitative assessment of small fiber neuropathy in patients receiving chemotherapy.

Chemotherapy-induced peripheral neurotoxicity (CIPN) is a common, potentially severe and dose-limiting adverse effect; however, it is poorly investigated at an early stage due to the lack of a simple assessment tool. As sweat glands are innervated by small autonomic C-fibers, sudomotor function testing has been suggested for early screening of peripheral neuropathy. This study aimed to evaluate Sudoscan, a non-invasive and quantitative method to assess sudomotor function, in the detection and follow-up of CIPN.

FXTAS: new insights and the need for revised diagnostic criteria.

Objective: Fragile X-associated tremor ataxia syndrome (FXTAS) is defined by FMR1 premutation, cerebellar ataxia, intentional tremor, and middle cerebellar peduncle (MCP) hyperintensities. We delineate the clinical, neurophysiologic, and morphologic characteristics of FXTAS.

Methods: Clinical, morphologic (brain MRI, (123)I-ioflupane SPECT), and neurophysiologic (tremor recording, nerve conduction studies) study in 22 patients with FXTAS, including 4 women.

Hereditary neuropathy with liability to pressure palsies occurring during military training.

Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal-dominant peripheral neuropathy characterized by recurrent isolated nerve palsies, which are precipitated by trivial compression and trauma. Although HNPP has been well-described in literature, it often goes unrecognized. We report a case of HNPP occurring during military training to promote recognition and proper management of this entity.

Severe meningo-radiculo-neuritis associated with ipilimumab.

Purpose: Ipilimumab is a T-cell-potentiating monoclonal antibody directed against cytotoxic T-lymphocyte antigen-4 (CTLA-4) to promote antitumoural immunity. In phase III trials, ipilimumab was shown to be the first agent to improve survival in advanced melanoma patients, regardless of previous treatment. We report a case of severe neurologic disease after ipilimumab treatment.

[Guillain-Barré syndrome and anti-hepatitis A vaccination].

The Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuritis. The pathophysiology remains unknown but the existence of triggering factors such as external antigens is regularly suspected. We report the case of a 30-year-old patient with a past medical history of Graves disease, who presented with GBS within the month after receiving an anti-hepatitis A vaccination.

[Tremor/ataxia syndrome related to Fragile X premutation].

The FXTAS syndrome (Fragile X-associated tremor/ataxia syndrome) is a specific neurodegenerative syndrome affecting subjects carrying a premutation of the FMR1 (fragile X mental retardation 1) gene. It affects mainly men with the premutation and aged more than 50 years. This syndrome is separate and distinct from the fragile X syndrome.

[Anticonvulsant hypersensitivity syndrome and lamotrigine-associated anticonvulsant hypersensitivity syndrome].

Anticonvulsant hypersensitivity syndrome (AHS) is defined by the association of high fever, cutaneous rash and multiorgan-system abnormalities (incidence, one in 1000 to one in 10,000 exposures). Fatal complications are described in 10%. This reaction usually develops 1 to 12 weeks after initiation of an aromatic anticonvulsant.

[Idiopathic intracranial hypertension as a side effect of doxycycline].

Idiopathic intracranial hypertension is a rare disorder characterized by elevated intracranial pressure without hydrocephaly or intracranial process. Its mechanism is poorly understood. Most cases of benign intracranial hypertension are presumed to be idiopathic but some of them may be related to some treatment.

[Interferon-beta retinopathy].

Introduction: Interferon-alpha associated retinopathy is an ocular complication of hepatitis C treatment well established in the literature. But, there are far fewer reports on multiple sclerosis related interferon-beta retinopathy.

Case Report: A 58-year-old male while receiving subcutaneous interferon-beta 1a 44microg thrice a week since 2001 for multiple sclerosis developed blurred vision.

[Neurological damage of brain tumor therapy].

Damage to the central nervous system induced by treatment of brain tumors is common and impairs the patient quality-of-life. Neurotoxicity is induced by synergistic effects of different cytotoxic treatments such as radiotherapy and chemotherapies administered concurrently or sequentially. Recent progress in the management of brain tumors has led to new neurotoxicities.

[Listeria monocytogenes abscess of the brain].

Introduction: Listeriosis commonly involves the central nervous system. Meningoencephalitis and rhomboencephalitis are the most frequent manifestations. Brain abscesses are rare.

[Spontaneous low cerebospinal fluid pressure syndrome. A case report and literature review].

Introduction: Spontaneous low cerebrospinal fluid pressure syndrome is a spontaneous intracranial hypotension pressure due to a cerebrospinal fluid leak without any known dural effraction. It is clinically characterised by postural headaches relieved by supine position. We report a 38-year-old patient with this syndrome and review the literature.

[Eulenburg's paramyotonia congenita].

Introduction: Paramyotonia congenita is an autosomal dominant sodium channelopathy, caused by mutations in gene coding for muscle voltage-gated sodium channel alpha subunit.

Case Report: We report the case of a 38-year-old man who described since childhood muscle stiffness with attacks ok weakness induced by two provocative stimuli: cold exposure and exercise. It primarily concerned eyelids and hands, occasionally limbs.