Measuring the diagnostic management and follow-up imaging for glioma patients across Belgian hospitals between 2016 and 2019.

Objectives: This study aimed to assess the diagnostic management and follow-up imaging for glioma patients across Belgian hospitals by calculating process indicators.

Methods: Patients with newly diagnosed glioma in Belgium (2016-2019) were selected from the Belgian Cancer Registry. The National Social Security Number served as unique patient identifier, linking the Registry to vital status and reimbursement data.

Life Expectancy and Causes of Death in Patients with Myotonic Dystrophy Type 2.

Background: Myotonic Dystrophy type 2 (DM2) is a dominantly inherited multisystem disease caused by a CCTG repeat expansion in intron 1 of the CNBP gene. Although in the last two decades over 1500 patients with DM2 have been diagnosed worldwide, our clinical impression of a reduced life expectancy in DM2 has not been investigated previously.

Objective: The aim of this observational study was to determine the life expectancy and the causes of death in patients with genetically confirmed DM2.

Child Neurology: Maternal Transmission of Congenital Myotonic Dystrophy Type 2: Case Report.

Congenital manifestations in Myotonic Dystrophy type 2 (DM2) point to anticipation and have only rarely been described. We report a three-generation family with genetically confirmed DM2. The youngest family member presented with unilateral congenital pes planovalgus and equinus.

Ancestral Origin of the First Indian Families with Myotonic Dystrophy Type 2.

Background: Myotonic dystrophy type 2 (DM2) is caused by a CCTG repeat expansion in intron 1 of the CCHC-Type Zinc Finger Nucleic Acid Binding Protein (CNBP) gene. Previous studies indicated that this repeat expansion originates from separate founders.

Objective: This study was set out to determine whether or not patients with DM2 originating from European and non-European countries carry the previously described European founder haplotypes.

High incidence of falls in patients with myotonic dystrophy type 1 and 2: A prospective study.

We aimed to examine the incidence as well as the circumstances and the consequences of falling in adult patients with myotonic dystrophy type 1 and 2 (DM1/DM2). We performed a prospective cohort study in 209 subjects, of which 102 had DM1, 42 had DM2 and 65 healthy controls. An assessment of their falls was carried out during 100 consecutive days.

First familial Becker muscular dystrophy in Tanzania: Clinical and genetic features.

In African neurological practice, muscle disorders are either underdiagnosed or underrepresented. This may in part be due to the large burden of other more common neurological disorders. In this report we describe the first Tanzanian patient with genetically confirmed Becker muscular dystrophy.

Graves' disease and celiac disease in a patient with myotonic dystrophy type 2.

We report a patient with progressive proximal muscle weakness in her legs, early-onset cataract and perceptive hearing loss, who was recently diagnosed with myotonic dystrophy type 2 (DM2). She also had two autoimmune disorders in her history, namely Graves' disease and celiac disease. Previous studies have shown a high frequency of autoimmune diseases (21%) in patients with DM2.

Qualitative and Quantitative Aspects of Pain in Patients With Myotonic Dystrophy Type 2.

Unlabelled: Pain is a common but often ignored symptom in patients with myotonic dystrophy type 2 (DM2). In this explorative study, we assessed qualitative and quantitative aspects of pain in DM2 using 4 questionnaires and quantitative sensory testing. A disease control group (fibromyalgia [FMS]) as well as healthy controls were used to compare the results, because pain in DM2 shows many clinical similarities to pain in FMS.

Hearing impairment in patients with myotonic dystrophy type 2.

Objective: To systematically assess auditory characteristics of a large cohort of patients with genetically confirmed myotonic dystrophy type 2 (DM2).

Methods: Patients with DM2 were included prospectively in an international cross-sectional study. A structured interview about hearing symptoms was held.

No relevant excess prevalence of myotonic dystrophy type 2 in patients with suspected fibromyalgia syndrome.

Myotonic dystrophy type 2 (DM2) is a rare, autosomal dominant, multisystem disorder with proximal weakness, myotonia, pain and cataract as important symptoms. Given the assumed underreporting of DM2 in the Netherlands combined with the predominant role of pain in DM2 as well as in fibromyalgia syndrome (FMS), we hypothesized there will be an excess prevalence of DM2 in patients with (suspected) FMS. Our objective was to determine the prevalence of DM2 in patients with suspected FMS.

PSEUDOTUMORAL TOPHACEOUS INVOLVEMENT OF THE ACHILLES PARATENON.

Gout is the most common form of microcrystalline arthropathy which usually does not pose a diagnostic challenge when patients have typical presentation, appropriate biochemical picture and classical radiographic appearance. However, formation of gouty tophi in unusual locations and with atypical presentations may mislead clinicians and radiologists, thereby justifying gout nickname as the "great mimicker". When interpreting images of tendon related masses, radiologists should be aware of gouty tophi as a possible differential given its variable and nonspecific imaging appearance.

Skeletal muscle involvement in myotonic dystrophy type 2. A comparative muscle ultrasound study.

This study determines the presence and extent of muscle changes in 31 myotonic dystrophy type 2 (DM2) patients detected by muscle ultrasound. Results were compared to 31 adult-onset myotonic dystrophy type 1 patients (DM1) and healthy controls. Furthermore, we tested the hypothesis that structural muscle changes correlate with age, quantitative muscle force and serum creatine kinase in both disorders.

High disease impact of myotonic dystrophy type 2 on physical and mental functioning.

The aim of the study was to investigate health status in patients with myotonic dystrophy type 2 (DM2) and determine its relationship to pain and fatigue. Data on health status (SF-36), pain (MPQ) and fatigue (CIS-fatigue) were collected for the Dutch DM2 population (n = 32). Results were compared with those of sex- and age-matched adult-onset myotonic dystrophy type 1 (DM1) patients.

Dutch myotonic dystrophy type 2 patients and a North-African DM2 family carry the common European founder haplotype.

Myotonic dystrophy type 2 (DM2) is a progressive multisystem disease with muscle weakness and myotonia as main characteristics. The disease is caused by a repeat expansion in the zinc-finger protein 9 (ZNF9) gene on chromosome 3q21. Several reports show that patients from European ancestry share an identical haplotype surrounding the ZNF9 gene.

Poor sleep quality and fatigue but no excessive daytime sleepiness in myotonic dystrophy type 2.

Background: In myotonic dystrophy type 1 (DM1), sleep disorders are common, with excessive daytime sleepiness (EDS) as a predominant feature. In myotonic dystrophy type 2 (DM2), the presence of sleep disturbances is unknown.

Objective: To investigate the frequency of EDS, poor sleep quality and fatigue in DM2.

Strong association between myotonic dystrophy type 2 and autoimmune diseases.

Background: Myotonic dystrophy type 2 (DM2) is a dominantly inherited multisystem disorder, characterised by progressive proximal weakness, myotonia, cataracts and cardiac conduction abnormalities. Our clinical impression of an association between DM2 and autoimmune diseases or autoantibody formation has not been published previously.

Objective: The aim of the present study was to investigate the frequency of autoimmune diseases and serum autoantibodies in patients with DM2 compared with patients with adult onset myotonic dystrophy type 1 (DM1).

Preoperative fMRI in tumour surgery.

Minimally invasive resection of brain tumours aims at removing as much pathological tissue as possible while preserving essential brain functions. Therefore, the precise spatial relationship between the lesion and adjacent functionally essential brain parenchyma needs to be known. Functional magnetic resonance imaging (fMRI) is increasingly being used for this purpose because of its non-invasiveness, its relatively high spatial resolution and the preoperative availability of the results.

Dysphagia is present but mild in myotonic dystrophy type 2.

The phenotype of myotonic dystrophy type 2 (DM2) shows similarities as well as differences to that of myotonic dystrophy type 1 (DM1). Dysphagia, a predominant feature in DM1, has not yet been examined in DM2. In a recent nationwide questionnaire survey of gastrointestinal symptoms in DM2, 12 out of 29 DM2 patients reported to have difficulty in swallowing for solid food.

Gastrointestinal involvement is frequent in Myotonic Dystrophy type 2.

The phenotype of DM2 shows similarities as well as differences to that of Myotonic Dystrophy type 1 (DM1). Gastrointestinal dysfunction is common in DM1 and 25% of the patients consider this to be the most disabling consequence of the disease. Little is known about gastrointestinal involvement in Myotonic Dystrophy type 2 (DM2).

Imaging of intralabyrinthine schwannomas: a retrospective study of 52 cases with emphasis on lesion growth.

Background And Purpose: Only a few case reports and small series of intralabyrinthine schwannomas (ILSs) have been reported. The purpose of this study was to assess prevalence, MR characteristics, location, clinical management, and growth potential/patterns of ILSs in the largest series reported.

Materials And Methods: Lesion localization, MR characteristics, lesion growth, and clinical management were reviewed in 52 patients diagnosed with an ILS between February 1991 and August 2007 in 2 referral centers.

Comparison between functional magnetic resonance imaging at 1.5 and 3 Tesla: effect of increased field strength on 4 paradigms used during presurgical work-up.

Objectives: We sought to evaluate the benefit of 3 T compared with 1.5 T during presurgical functional magnetic resonance imaging.

Materials And Methods: Six participants performed a motor, a visual, and 2 language paradigms both at 1.

Lateralized anterior mesiotemporal lobe activation: semirandom functional MR imaging encoding paradigm in patients with temporal lobe epilepsy--initial experience.

Purpose: To prospectively demonstrate anterior mesiotemporal lobe (MTL) activation in healthy volunteers by using a semirandom memory-encoding paradigm and to prospectively compare lateralized functional magnetic resonance (MR) imaging activation with intracarotid amobarbital procedure (IAP) memory test results in patients with temporal lobe epilepsy (TLE) who were scheduled to undergo surgery.

Materials And Methods: The study was approved by a local ethics committee, and written informed consent was obtained from all subjects. Eight healthy volunteers and 18 patients with TLE who were scheduled for surgery were included in the functional MR imaging study involving the use of a memory-encoding paradigm with variable epoch lengths.

Stimulus pacing affects the activation of the medial temporal lobe during a semantic classification task: an fMRI study.

Our purpose was to explore the influence of stimulus pacing in blocked functional MRI studies on the activation pattern elicited by a semantic retrieval task. Twenty-two participants performed both a fixed-paced and a self-paced functional MR imaging experiment in which a semantic categorization (animal/object) task was contrasted with a perceptual (small/capital letter string) categorization task. Group and single-subject ROI analyses were computed.