Sickle cell disease mice have cerebral oxidative stress and vascular and white matter abnormalities.

Strokes are feared complications of sickle cell disease (SCD) and yield significant neurologic and neurocognitive deficits. However, even without detectable strokes, SCD patients have significant neurocognitive deficits in domains of learning and memory, processing speed and executive function. In these cases, mechanisms unrelated to major cerebrovascular abnormalities likely underlie these deficits.

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.

Effect of 810 nm light on nerve regeneration after autograft repair of severely injured rat median nerve.

Background And Objective: Destruction of large segments of peripheral nerves results in chronic loss of sensation and paralysis. For this type of severe injury, the defect can be bridged by nerve grafts. However, even with state-of-the-art microsurgical techniques, there is minimal recovery of sensation and motor function.

Light therapy and supplementary Riboflavin in the SOD1 transgenic mouse model of familial amyotrophic lateral sclerosis (FALS).

Background And Objective: Familial amyotrophic lateral sclerosis (FALS) is a neurodegenerative disease characterized by progressive loss of motor neurons and death. Mitochondrial dysfunction and oxidative stress play an important role in motor neuron loss in ALS. Light therapy (LT) has biomodulatory effects on mitochondria.

Sensory physiology assessed by evoked potentials in survivors of poliomyelitis.

Evidence suggests that sensory loss may occur in a proportion of patients affected by poliomyelitis. We hypothesize that sensory problems may be a lasting sequela in some polio survivors. Sensory pathways in polio survivors were evaluated clinically and electrophysiologically using sensory evoked potentials (SEPs).

Physiology of the motor cortex in polio survivors.

We hypothesized that the corticospinal system undergoes functional changes in long-term polio survivors. Central motor conduction times (CMCTs) to the four limbs were measured in 24 polio survivors using transcranial magnetic stimulation (TMS). Resting motor thresholds and CMCTs were normal.

Potential outcome factors in subacute combined degeneration: review of observational studies.

Background: Subacute combined degeneration is an acquired myelopathy caused by vitamin B12 deficiency. Therapy with B12 leads to improvement in most but to complete recovery in only a few patients. Prognostic indicators in subacute combined degeneration are unknown; therefore, predicting complete recovery of neurologic deficits is challenging.

A comparison of fatigue scales in postpoliomyelitis syndrome.

Objective: To examine the applicability and validity of traditional fatigue questionnaires in postpoliomyelitis syndrome (PPS) patients with disabling fatigue.

Design: Cross-sectional study. PPS and disabling fatigue were ascertained according to published criteria.

Dermatomyositis-like syndrome and HMG-CoA reductase inhibitor (statin) intake.

A patient developed an adult-onset dermatomyositis-like syndrome characterized by skin rash and progressive proximal muscle weakness concurrent with the intake of simvastatin. Despite discontinuation of the statin, symptoms progressed and required conventional steroid therapy for remission. The association between statins and the development of a musculocutaneous syndrome closely resembling dermatomyositis in susceptible subjects is poorly understood and has been reported rarely.

Stiff-person Syndrome.

Stiff-person syndrome (SPS) is a progressive neurologic disorder characterized by 1) stiffness that is prominent in axial muscles, with co-contraction of agonist and antagonist muscles; 2) sudden episodic spasms; and 3) absence of another disease that causes similar symptoms. The diagnosis of SPS is based on clinical grounds and requires a high degree of suspicion. The diagnosis is, however, aided by electromyography, which demonstrates motor unit firing at rest simultaneously from the agonist and antagonist muscles, and by high serum titers of antibodies against glutamic acid decarboxylase (GAD), the rate-limiting enzyme for the synthesis of gamma-aminobutyric acid (GABA), which is the brain's main inhibitory neurotransmitter.

GNE mutations in an American family with quadriceps-sparing IBM and lack of mutations in s-IBM.

Analysis for GNE mutations was performed in an American, non-Iranian Jewish, family with quadriceps-sparing inclusion body myopathy (QS-IBM) and in 11 patients with sporadic IBM (s-IBM). Two novel nonallosteric site missense mutations were found in the QS-IBM kinship. No mutations were identified in s-IBM patients.