Gastrointestinal and Urologic Sphincter Dysfunction in Stiff Person Syndrome.

Objectives: Stiff person syndrome is a neurologic disorder characterized by axial rigidity leading to progressive disability, with broad clinical spectrum.

Methods: We report 2 cases with unique clinical presentation.

Results: Two young men suffered progressive urinary retention requiring bladder catheterization, anorectal spasms and constipation, complicated subsequently with lower extremity trigger-induced spasms, and gait instability.

Sodium nitrate alleviates functional muscle ischaemia in patients with Becker muscular dystrophy.

Becker muscular dystrophy (BMD) is a progressive X-linked muscle wasting disease for which there is no treatment. BMD is caused by in-frame mutations in the gene encoding dystrophin, a structural cytoskeletal protein that also targets other proteins to the sarcolemma. Among these is neuronal nitric oxide synthase mu (nNOSμ), which requires specific spectrin-like repeats (SR16/17) in dystrophin's rod domain and the adaptor protein α-syntrophin for sarcolemmal targeting.

The elbow flex-ex: a new sign to detect unilateral upper extremity non-organic paresis.

Objective: To examine a new neurological sign that uses synergistic oppositional movements of the arms to evaluate for non-organic upper extremity weakness.

Methods: Patients with unilateral arm weakness were tested in a standing or sitting position with the elbows flexed at 30°. The examiner held both forearms near the wrists while asking the patient to flex or extend the normal arm at the elbow and simultaneously feeling for flexion or extension of the contralateral (paretic) arm.

Tadalafil alleviates muscle ischemia in patients with Becker muscular dystrophy.

Becker muscular dystrophy (BMD) is a progressive X-linked muscle wasting disease for which there is no treatment. Like Duchenne muscular dystrophy (DMD), BMD is caused by mutations in the gene encoding dystrophin, a structural cytoskeletal protein that also targets other proteins to the muscle sarcolemma. Among these is neuronal nitric oxide synthase (nNOSμ), which requires certain spectrin-like repeats in dystrophin's rod domain and the adaptor protein α-syntrophin to be targeted to the sarcolemma.