Presentation and Outcome in S1P-RM and Natalizumab-Associated Progressive Multifocal Leukoencephalopathy: A Multicenter Cohort Study.

Background And Objectives: Progressive multifocal leukoencephalopathy (PML) is a severe neurologic disease resulting from JC virus reactivation in immunocompromised patients. Certain multiple sclerosis (MS) disease-modifying therapies (DMTs) are associated with PML risk, such as natalizumab and, more rarely, sphingosine-1-phosphate receptor modulators (S1P-RMs). Although natalizumab-associated PML is well documented, information on S1P-RM-associated PML is limited.

Profile of precipitating factors and its implication in 160 Indian patients with Moyamoya angiopathy.

Introduction: Moyamoya angiopathy (MMA) has been known to manifest with myriad of neurological manifestations, often in association with various precipitating factors. This is the first study to systematically analyze the precipitating triggers to neurological symptoms done on the largest cohort of MMA in India.

Methods: A single-centered, cross-sectional observational study, recruiting 160 patients with consecutive angiographically proven MMA over a period of 5 years (2016-2021), was undertaken to evaluate the profile of immediate precipitating factors in temporal association to the neurological symptoms, along with their clinical and radiological characteristics.

The angiographic presentation of European Moyamoya angiopathy.

Background And Purpose: Little is known about the angiographic presentation of Moyamoya angiopathy (MMA) in non-Asian patients.

Methods: Conventional cerebral angiograms from 155 Caucasian patients diagnosed as MMA were analyzed with respect to extracranial champagne bottle neck sign, Suzuki stages, collateral status, as well as presence of aneurysms and posterior cerebral artery stenosis.

Results: In 84 of 155 angiograms, the extracranial carotid artery was visualized, in 65 of them (77.

Cutaneous manifestations in Moyamoya angiopathy: A review.

Background And Purpose: Moyamoya angiopathy (MA) is a progressive cerebrovascular disease with a poorly understood pathophysiology. It is mainly characterized by progressive bilateral stenosis of the terminal intracranial part of the supraclinoid internal carotid arteries and the proximal parts of the middle and anterior cerebral arteries. This results in early-onset ischemic or hemorrhagic strokes.

The disease presentation of Moyamoya angiopathy in Eastern India.

Introduction: Clinical spectrum of Moyamoya angiopathy (MMA) differs across populations with different ethnicity. This study, the largest one done among Indian population was undertaken to assess clinico-radiological profile of MMA patients in eastern India.

Methods: A single centre cross-sectional study was undertaken among 76 MMA cases.

Livedo racemosa in neurological diseases: an update on the differential diagnoses.

Livedo is a net-like violaceous skin pattern. It can be classified as physiological or pathological. The physiological livedo reticularis usually appears in cold conditions, whereas the pathological and irregular livedo, which persists in warm temperatures, is often labeled as 'livedo racemosa'.

Thalassemia and Moyamoya syndrome: unfurling an intriguing association.

Introduction: Moyamoya angiopathy (MMA) is a rare cerebrovascular disease with progressive bilateral narrowing of intracranial parts of the internal carotid artery and proximal parts of the anterior and middle cerebral artery resulting in recurrent hemodynamic ischemic attacks, strokes and hemorrhages. If associated with other diseases, it is called Moyamoya syndrome (MMS). Until now, MMS has rarely been described with thalassemia.

Extensive immune reconstitution inflammatory syndrome in Fingolimod-associated PML: a case report with 7 Tesla MRI data.

Background: Progressive multifocal leukoencephalopathy (PML) is a rare complication of patients treated with fingolimod.

Case Presentation: Routine MRI eventually led to diagnosis of asymptomatic early PML that remained stable after discontinuation of fingolimod. As blood lymphocyte counts normalized, signs of immune reconstitution inflammatory syndrome (IRIS) and renewed MS activity developed.

Moyamoya angiopathy in PHACE syndrome not associated with RNF213 variants.

Moyamoya angiopathy is a rare vasculopathy with stenosis and/or occlusion of bilateral intracranial parts of internal carotid arteries and/or proximal parts of middle and anterior cerebral arteries. PHACE syndrome is characterized by large segmental hemangiomas in the cervical-facial region. Both conditions are known to be associated in rare cases.

Efficacy of STA-MCA bypass surgery in moyamoya angiopathy: long-term follow-up of the Caucasian Krupp Hospital cohort with 81 procedures.

Background: Despite the consensus on the efficacy of revascularizing surgery in moyamoya angiopathy (MA) in Asia, the indication in Caucasian moyamoya patients is controversially discussed.

Objective: The efficacy of revascularizing surgery in adult European patients with MA should be clarified.

Methods: This study retrospectively analyzed the rate of further strokes and hemorrhages as well as MRI and Duplex ultrasound features during long-term follow up after STA-MCA bypass.

Association of primary central nervous system vasculitis with the presence of specific human leucocyte antigen gene variant.

Objectives: The etiology and genetic susceptibility of primary central nervous system vasculitis (PCNSV) are still unclear.

Patients And Methods: We analyzed the DNA of 25 Caucasian patients with PCNSV for human leucocyte antigen genes HLA-A, HLA-B, HLA-DRB1, and HLA-DQB1, respectively. HLA-frequencies of the 25 patients with PCNSV were compared with HLA-frequencies of matched Caucasian controls.

Primary central nervous system vasculitis and its mimicking diseases - clinical features, outcome, comorbidities and diagnostic results - A case control study.

Objectives: To compare clinical features and outcome, imaging characteristics, biopsy results and laboratory findings in a cohort of 69 patients with suspected or diagnosed primary central nervous system vasculitis (PCNSV) in adults; to identify risk factors and predictive features for PCNSV.

Patients And Methods: We performed a case-control-study including 69 patients referred with suspected PCNSV from whom 25 were confirmed by predetermined diagnostic criteria based on biopsy (72%) or angiography (28%). Forty-four patients turned out to have 15 distinct other diagnoses.

Movement symptoms in European Moyamoya angiopathy - First systematic questionnaire study.

Objective: Movement disorders are a rare manifestation of Moyamoya angiopathy (MMA). Data on prevalence and clinical presentation are warranted. Possible involuntary movements include focal motor seizures, tremor, limb-shaking transient ischemic attacks, choreiform and spastic or dystonic movement disorders.

Improved cerebral time-of-flight magnetic resonance angiography at 7 Tesla--feasibility study and preliminary results using optimized venous saturation pulses.

Purpose: Conventional saturation pulses cannot be used for 7 Tesla ultra-high-resolution time-of-flight magnetic resonance angiography (TOF MRA) due to specific absorption rate (SAR) limitations. We overcome these limitations by utilizing low flip angle, variable rate selective excitation (VERSE) algorithm saturation pulses.

Material And Methods: Twenty-five neurosurgical patients (male n = 8, female n = 17; average age 49.

Theory of mind and empathy in patients at an early stage of relapsing remitting multiple sclerosis.

Introduction: Early after having been diagnosed with relapsing remitting multiple sclerosis (RRMS), young patients coping with the new situation require good social support and interactions. Successful social interaction is critically dependent upon the ability to understand the minds of others and their feelings. Social cognition refers to the ability to understand the mind of others.

Perception of affective prosody in patients at an early stage of relapsing-remitting multiple sclerosis.

Cognitive dysfunction is well known in patients suffering from multiple sclerosis (MS) and has been described for many years. Cognitive impairment, memory, and attention deficits seem to be features of advanced MS stages, whereas depression and emotional instability already occur in early stages of the disease. However, little is known about processing of affective prosody in patients in early stages of relapsing-remitting MS (RRMS).

Differential diagnosis between cerebral ischemia, focal seizures and limb shaking TIAs in moyamoya disease.

We describe the clinical course of a young female Caucasian patient with bilateral moyamoya disease in whom we could diagnose the simultaneous occurrence of cerebral ischemia, TIAs, limb shaking TIAs and focal Jacksonian seizures. It is the second clinical communication in the literature elaborating limb shaking TIAs in moyamoya disease.

Analysis of human leucocyte antigen genes in Caucasian patients with idiopathic moyamoya angiopathy.

Background: The etiology and genetic susceptibility of Moyamoya angiopathy (MMA) (Moyamoya disease, Moyamoya syndrome and unilateral type of MMA) still remain unclear. In Asian patient cohorts several HLA markers were described to be associated with MMA, but in Caucasians very little is known about genetic susceptibility of this angiopathy.

Method: We analysed DNA of 33 Caucasian patients with MMA for HLA-A, HLA-B, HLA-DRB1, and HLA-DQB1 markers, respectively.

Inheritance of moyamoya disease in a Caucasian family.

Background And Purpose: Moyamoya disease is a very rare occlusive cerebrovascular disorder characterized by progressive stenosis or occlusion of the intracranial portion of the internal carotid artery and proximal cerebral arteries with an extensive network of fine collaterals. The aetiology and genetic susceptibility of moyamoya disease, especially in Caucasians, still remains unclear.

Methods And Results: We describe the cases of affected German father, daughter and son with juvenile stroke because of idiopathic moyamoya disease.

Reduction in jaw opening: a neglected symptom of giant cell arteritis.

Reduction in jaw opening is a neglected symptom of giant cell arteritis (GCA) in clinical practice and in the scientific literature. We describe the case of a 71-year-old woman with GCA who was misdiagnosed as occipital neuritis and craniomandibular dysfunction because of headaches in the occipital region and reduction in jaw opening. The reported case reminds us not to overlook reduction in jaw opening as a symptom to reveal GCA in elderly patients.

Activation of working memory in patients at the earliest stage of multiple sclerosis--an fMRI study.

Objective: Previous fMRI studies on activation of working memory in multiple sclerosis (MS) patients presented heterogeneous results. Patients were reported to have altered brain activation patterns either in typical working memory circuits or in other brain regions even if they did not have any cognitive impairment according to the test batteries. We hypothesized that brain activation patterns in patients at a very early stage of the disease at very low EDSS-Score would not differ from healthy subjects.

Primary central nervous system vasculitis and moyamoya disease: similarities and differences.

Primary central nervous system vasculitis (PCNSV) and moyamoya disease (MMD) represent rare and poorly-understood causes of stroke. Both may present with similar clinical and auxiliary findings, but differentiation is extremely important because they require different treatment regimens. Our cohort included 21 white patients with PCNSV and 21 white patients with MMD.

Primary central nervous system vasculitis: clinical experiences with 21 new European cases.

Primary central nervous system vasculitis (PCNSV) represents a rare and poorly understood form of vascular inflammatory disease restricted to the brain and spinal cord. This study was undertaken to better characterize demographic and clinical features and to evaluate treatments and outcome. Our cohort included 21 white patients with PCNSV treated in a single German institution between 2003 and 2008.

Diagnostic problems and delay of diagnosis in amyotrophic lateral sclerosis.

Objective: Initial symptoms of amyotrophic lateral sclerosis (ALS) mimic several neurological syndromes that may decelerate a correct diagnosis. The aim of our study was to investigate if diagnostic and therapeutic parameters have influence on the time of diagnosis.

Methods: We retrospectively reviewed the medical records of 100 consecutive ALS patients focusing on clinical and diagnostic data, the timing of diagnosis and treatments attributed to the onset of symptoms of ALS.