A double-blind, placebo-controlled study of rituximab in patients with stiff person syndrome.

Objective: In stiff person syndrome (SPS), an antibody-mediated impaired γ-aminobutyric acidergic (GABAergic) neurotransmission is believed to cause muscle stiffness and spasms. Most patients improve with GABA-enhancing drugs and intravenous immunoglobulin, but some respond poorly and remain disabled. The need for more effective therapy prompted a trial with the anti-CD20 monoclonal antibody rituximab.

Reliability of the adult myopathy assessment tool in individuals with myositis.

Objective: The Adult Myopathy Assessment Tool (AMAT) is a 13-item performance-based battery developed to assess functional status and muscle endurance. The purpose of this study was to determine the intrarater and interrater reliability of the AMAT in adults with myositis.

Methods: Nineteen raters (13 physical therapists and 6 physicians) scored videotaped recordings of patients with myositis performing the AMAT for a total of 114 tests and 1,482 item observations per session.

Lower extremity peak force and gait kinematics in individuals with inclusion body myositis.

Objective: To determine the relationship between peak isometric muscle force and temporal characteristics of gait in individuals with sporadic inclusion body myositis (s-IBM).

Methods: An observational study of 42 individuals with s-IBM (12 women; mean ± SD age 61.8 ± 7.

Validity of the single limb heel raise test to predict lower extremity disablement in patients with sporadic inclusion body myositis.

Purpose: To determine the validity of the single limb heel raise (SLHR) test as a potential screening tool to detect lower extremity disability in patients with sporadic inclusion body myositis (sIBM).

Methods: We compared gait speed and fall history between subjects with sIBM who either could complete one SLHR (SLHR group) or could not complete one SLHR. Discriminative validity was established by comparing between group differences in functional measures based on group assignment.

Are repeated single-limb heel raises and manual muscle testing associated with peak plantar-flexor force in people with inclusion body myositis?

Background: Repeated heel raises have been proposed as a method of ankle plantar-flexor strength testing that circumvents the limitations of manual muscle testing (MMT).

Objective: The study objective was to examine the relationships among ankle plantar-flexion isometric maximum voluntary contraction (MVC), repeated single-limb heel raises (SLHRs), and MMT in people with myositis.

Design: This was a cross-sectional study with a between-group design.

Effect of Alemtuzumab (CAMPATH 1-H) in patients with inclusion-body myositis.

Sporadic inclusion-body myositis (sIBM) is the most common disabling, adult-onset, inflammatory myopathy histologically characterized by intense inflammation and vacuolar degeneration. In spite of T cell-mediated cytotoxicity and persistent, clonally expanded and antigen-driven endomysial T cells, the disease is resistant to immunotherapies. Alemtuzumab is a humanized monoclonal antibody that causes an immediate depletion or severe reduction of peripheral blood lymphocytes, lasting at least 6 months.

Placebo-controlled trial of rituximab in IgM anti-myelin-associated glycoprotein antibody demyelinating neuropathy.

Objective: Report a double-blind, placebo-controlled study of rituximab in patients with anti-MAG demyelinating polyneuropathy (A-MAG-DP).

Methods: Twenty-six patients were randomized to four weekly infusions of 375 mg/m(2) rituximab or placebo. Sample size was calculated to detect changes of > or = 1 Inflammatory Neuropathy Course and Treatment (INCAT) leg disability scores at month 8.

Gender-related differences in the clinical presentation of malignant and benign pheochromocytoma.

Signs and symptoms associated with pheochromocytomas are predominantly caused by catecholamine excess, but tend to be highly variable and non-specific. In this study, we evaluated 23 male and 35 female pheochromocytoma patients for symptoms and signs of pheochromocytoma with special regard to gender-related differences in presentation. Total symptom score comparison between genders showed significant differences (12.

Superiority of 6-[18F]-fluorodopamine positron emission tomography versus [131I]-metaiodobenzylguanidine scintigraphy in the localization of metastatic pheochromocytoma.

The purpose of the study was to assess the diagnostic utility of 6-[(18)F]-fluorodopamine ([(18)F]-DA) positron emission tomography scanning (PET) vs. [(131)I]-metaiodobenzylguanidine (MIBG) scintigraphy in patients with metastatic pheochromocytoma (PHEO). We studied 10 men and six women (mean age 38.