Prevention of axonal loss after immediate dosage titration of immunoglobulin in multifocal motor neuropathy.

Background: To evaluate whether ongoing axonal loss can be prevented in multifocal motor neuropathy (MMN) treated with immunoglobulin G (IgG), a group of patients with a median disease duration of 15.7 years (range: 8.3-37.

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CANVAS including its clinical components of cerebellar ataxia, sensory neuropathy and vestibular areflexia is presented in this review. An intronic biallelic pentanucleotide expansion in RFC1 is the genetic cause of CANVAS. Several patients diagnosed with isolated "idiopathic" neurological or otological conditions might have a CANVAS spectrum disorder.

Outcome in chronic inflammatory demyelinating polyneuropathy: A systematic review and meta-analysis.

Introduction/aims: Outcomes in chronic inflammatory demyelinating polyneuropathy (CIDP) have been reported in longitudinal and cross-sectional studies. A considerable variation in long-term disease outcome has appeared in those reports. To overcome this uncertainty, a systematic review and meta-analysis was conducted on CIDP outcomes, including the parameters of case fatality rate, ambulation, physical ability, and remission.

Axonal loss at time of diagnosis as biomarker for long-term disability in chronic inflammatory demyelinating polyneuropathy.

Introduction/aims: We hypothesized that early, pretreatment axonal loss would predict long-term disability, supported by a pilot study of selected patients with chronic inflammatory demyelinating polyneuropathy (CIDP). To further test this hypothesis, we examined a larger consecutive group of CIDP patients.

Methods: Needle electromyography and motor and sensory nerve conduction studies were carried out in 30 CIDP patients at pretreatment and follow-up 5 to 28 years later.

Long-term follow-up of facilitated subcutaneous immunoglobulin therapy in multifocal motor neuropathy.

Objective: To assess the feasibility, efficacy and patient satisfaction of long-term facilitated subcutaneous immunoglobulin therapy (fSCIG) in multifocal motor neuropathy (MMN).

Methods: Twelve patients previously participating in a randomized trial investigating the short-term efficacy of fSCIG were offered to switch to fSCIG maintenance therapy following a variable interval on conventional subcutaneous immunoglobulin.

Results: Eight patients were switched to fSCIG maintenance therapy, seven of whom were invited for a follow-up assessment after 18 months (range 13-23 months) of treatment.

Early axonal loss predicts long-term disability in chronic inflammatory demyelinating polyneuropathy.

Objective: To investigate early pre-treatment nerve fiber loss as a predictor of long-term clinical outcome in chronic inflammatory demyelinating polyneuropathy (CIDP).

Methods: In 14 patients, motor and sensory conduction studies of the median, fibular, and sural nerves were performed at pre-treatment and follow-up 11-28 years later. Z-scores of amplitudes were combined as biomarkers of axonal loss and Z-scores of conduction properties as demyelination scores.

A population-based study of long-term outcome in treated chronic inflammatory demyelinating polyneuropathy.

Introduction: The effect of long-lasting immune-modulating therapy was studied in patients with chronic inflammatory demyelinating polyneuropathy (CIDP).

Methods: A population-based, cross-sectional study of treated patients referred to the Danish health-care system between 1985 and 2006.

Results: The 51 participating patients had a median disease duration of 16 (interquartile range, 14-21) years.

[Chronic inflammatory demyelinating polyneuropathy].

In this review, we discuss chronic inflammatory demyelinating polyneuropathy (CIDP), which is a disease with proximal and distal weakness and sensory disturbances resulting in impaired daily activity. The diagnosis is based on the clinical presentation and electrophysiology demonstrating demyelination in the peripheral nerves. CIDP can be successfully treated with immunoglobulin, glucocorticoids or plasma exchange, and during the latest decade, immunoglobulin has been administered subcutaneously improving patients' flexibility and autonomy.

A population-based and cross-sectional study of the long-term prognosis in multifocal motor neuropathy.

A population-based, cross-sectional study of patients referred to the Danish hospital system between 1985 and 2006 was conducted to evaluate the long-term outcome in Danish patients treated for multifocal motor neuropathy (MMN). Thirty-four MMN patients were identified, three had died of unrelated diseases, 10 were excluded, one did not reply to study request and 20 were included. The median disease duration was 24 years (interquartile range: 18.

Muscle weakness and functional disability in patients with myasthenia gravis.

Introduction: Muscle weakness and functional disability have not been evaluated in a population-based study of patients with myasthenia gravis (MG).

Methods: All patients with MG in a well-defined catchment area were identified in the Danish National Patient Registry. Of the 175 eligible patients, 90 participated and were studied using MG-specific scales, isometric dynamometry, and functional tests.

Comparisons in fluctuation of muscle strength and function in patients with immune-mediated neuropathy treated with intravenous versus subcutaneous immunoglobulin.

Introduction: Variations in muscle strength and function have not been studied in patients with chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy whose treatment regimen has been changed from intravenous to subcutaneous immunoglobulin (IVIg to SCIg).

Methods: In a prospective, open-label study, patients were changed from monthly IVIg to weekly SCIg. The primary endpoint was variation in isokinetic muscle strength (cIKS).

Quantitative sensory testing and structural assessment of sensory nerve fibres in amyotrophic lateral sclerosis.

Objective: In this prospective study, involvement of sensory nerve fibres in ALS patients was assessed using functional and structural measures in the form of quantitative sensory testing (QST) and skin and nerve biopsies.

Methods: Thirty-two ALS patients and 32 healthy subjects were evaluated with a QST battery comprising thresholds of mechanical detection, mechanical pain, vibration detection, cold detection, warm detection, heat pain, and pinprick sensation. Skin biopsies were evaluated in 31 ALS patients by intraepidermal nerve fibre density (IENFD) and axonal swelling ratios, and growth-associated protein 43 (GAP-43) antibody staining.

Improvement of hemoglobin levels after a switch from intravenous to subcutaneous administration of immunoglobulin in chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy.

Background: Intravenous immunoglobulin (IVIG) is recommended treatment for chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). Recent studies have demonstrated that subcutaneous immunoglobulin (SCIG) is feasible, safe, and effective in both disorders. IVIG leads to transient hemolysis and, consequently, we hypothesized that frequent small doses of SCIG exerts less hemolytic activity than a few larger doses of IVIG.

Skeletal muscle fiber characteristics and oxidative capacity in hemiparetic stroke survivors.

Introduction: Skeletal muscle is changed after stroke, but conflicting data exist concerning muscle morphology and oxidative enzyme capacity.

Methods: In 36 chronic stroke patients bilateral rectus femoris muscle biopsies were analyzed, and fiber type proportions and cross-sectional areas were determined by ATPase histochemistry. Enzymatic concentrations of citrate synthase (CS) and 3-Hydroxyacyl-coenzymeA-dehydrogenase (HAD) were determined using freeze-dried muscle tissue.

Daily Life Experiences of Patients With a High-Grade Glioma and Their Caregivers: A Longitudinal Exploration of Rehabilitation and Supportive Care Needs.

Background: There is a lack of knowledge regarding the breadth of needs for rehabilitation and supportive care across the disease and treatment trajectory for patients with a high-grade glioma (HGG) and their caregivers.

Objective: The aim of this study was to elucidate the experiences and needs for rehabilitation and supportive care in patients with HGG and their caregivers.

Methods: Patients with malignant glioma (N = 30) and their caregivers (N = 33) were interviewed five times during the first year of the HGG trajectory.

Upper Respiratory Infections and MRI Activity in Relapsing-Remitting Multiple Sclerosis.

Background: Although clinical reports have suggested a relationship between systemic infections and multiple sclerosis (MS) relapses, MRI evidence supporting an association is conflicting. Here we evaluated the temporal relationship between upper respiratory infections (URIs) and MRI activity in relapsing-remitting (RR) MS.

Methods: We combined individual data on URI with data on active lesions in pre-scheduled MRI examinations performed every 4 weeks for 28 weeks in 69 patients.

Headache and Nausea after Treatment with High-Dose Subcutaneous versus Intravenous Immunoglobulin.

Treatment with intravenous immunoglobulin (IVIG) leads to transient side effects such as headache and nausea during and after the infusion. We hypothesized that subcutaneous administration of smaller doses of immunoglobulin (SCIG) given more frequently leads to less severe headache and nausea and could be an alternative in patients experiencing side effects. Fifty-nine patients diagnosed with neurological disorders (chronic inflammatory demyelinating polyneuropathy (CIDP), multi-focal motor neuropathy (MMN) or post-polio syndrome) were treated with IVIG, and 27 CIDP or MMN patients with SCIG.

Mortality in myasthenia gravis: A nationwide population-based follow-up study in Denmark.

Introduction: In previous studies of myasthenia gravis (MG), increased mortality has been reported. The aim of this study was to estimate mortality in patients with acetylcholine receptor antibody-positive (AChR-Ab-seropositive) MG in a nationwide population-based, long-term follow-up study.

Methods: All AChR-Ab-seropositive MG patients, diagnosed between 1985 and 2005, were identified.

Diurnal and day-to-day variation of isometric muscle strength in myasthenia gravis.

Introduction: In patients with myasthenia gravis (MG), muscle strength is expected to decrease gradually during the day due to physical activities.

Methods: Isometric muscle strength at the shoulder, knee, and ankle was determined in 10 MG patients (MGFA class II-IV) who were receiving usual medical treatment and in 10 control subjects. To determine diurnal and day-to-day variation, muscle strength was measured 4 times during day 1 and once at day 2.

[Acute kidney injury secondary to the first intravenous administration of acyclovir].

Acute kidney injury secondary to precipitation of acyclovir crystals in the kidneys is well known and mainly observed in the setting of dehydration or pre-existing renal impairment. We describe a case of acute kidney injury secondary to intravenous administration of acyclovir in a 64-year-old female with trans-versal myelitis and no prior medical history. She developed a rapid rise in the plasma creatinine level only seven hours after the primary drug administration.

Striated muscle fiber size, composition, and capillary density in diabetes in relation to neuropathy and muscle strength.

Objective: Diabetic polyneuropathy (DPN) leads to progressive loss of muscle strength in the lower extremities due to muscular atrophy. Changes in vascularization occur in diabetic striated muscle; however, the relationship between these changes and DPN is as yet unexplored. The aim of the present study was to evaluate histologic properties and capillarization of diabetic skeletal muscle in relation to DPN and muscle strength.

Effects of resistance training and aerobic training on ambulation in chronic stroke.

Objective: The aim of this study was to directly compare the effects of aerobic training (AT) with progressive resistance training (RT) after stroke to determine whether AT-induced fitness gains or RT-induced strength gains translate into improved ambulation across a 12-wk intervention and whether gains are retained 1 yr after cessation of formal training.

Design: This study is a randomized controlled 12-wk intervention trial with a 1-yr follow-up. Forty-three community-dwelling independent walkers with a chronic ischemic hemiparetic stroke were allocated to AT using a cycle ergometer (n = 13), RT using training machines (n = 14), or low-intensity sham training of the arms (n = 16).

A novel de novo mutation of the mitochondrial tRNAlys gene mt.8340G>a associated with pure myopathy.

Most patients with mutations in the tRNA(lys) gene (MTTK) present with symptoms from the central nervous system (CNS). We describe a 41-year-old woman with pure myopathy associated with a novel de novo mtDNA mutation, mt.8340G>A, which was heteroplasmic in muscle (53%), blood, urine and mouth epithelial cells (<7%).

Lower muscle endurance in patients with alcoholic liver disease.

Patients with alcoholic liver disease often complain of restricted physical capacity, which could be due to decreased muscle endurance. The aim of this study was to assess the muscular endurance in patients with alcoholic liver disease. In a cross sectional study, 24 patients with alcoholic liver disease and 22 controls were evaluated using isometric dynamometry.

[Exfoliative dermatitis as a side effect of intravenous immunoglobulin treatment].

Three patients with immune-mediated polyneuropathies developed rash, eczema, whole body scaling, vesicles in hands and loss of hair a few days after infusion of large doses of intravenous immunoglobulin (IVIG). The condition was diagnosed as exfoliative dermatitis. Two out of three patients were afterwards treated with low doses of IVIG slowly increased over a year given under the protection of oral steroids.