Mark Sivak

ALSFRS AND APPEL ALS SCORES : DISCORDANCE WITH DISEASE PROGRESSION

Progression of disease and effectiveness of therapy in patients with amyotrophic lateral sclerosis (ALS) are determined by both questionnaire‐ and examination‐based measures. To determine whether both types of measurement tools are equally predictive at all stages of disease, we compared questionnaire‐based ALS Functional Rating Scale (ALSFRS) scores to the examination‐based Appel ALS (AALS) scores at different stages of disease. Same‐day scores were obtained during 174 visits in 62 patients with definite or probable ALS. Using normalized scores, correlation between the scales and predictability were best in mildly affected patients. Predictions of ALSFRS based on AALS scores were less than half as precise in the later stages of disease. Both scales showed significant change with disease progression, but ALSFRS consistently underestimated disease severity defined by AALS (P < 0.001). Questionnaire‐based measurements should be compared against objective scales at all stages of disease severity before they are accepted as primary endpoint measures. Muscle Nerve, 2008

Single‐fiber electromyography shows terminal axon dysfunction in Miller Fisher syndrome: A case report

We studied a patient with ophthalmoparesis and pupillary areflexia 2 weeks after a viral syndrome. Miller Fisher syndrome was suspected but GQ1b antibodies were not detected. To define neuromuscular involvement we performed electrodiagnostic studies. Single‐fiber electromyography (SFEMG) in the extensor digitorum communis (EDC) showed abnormal jitter and axonal blocking, suggesting terminal axon dysfunction. Subsequent GQ1b antibody titers were elevated to borderline levels. Clinical symptoms gradually resolved. SFEMG may help characterize neuropathies associated with antibodies to neuronal ganglioside and identify involvement of the terminal axon and neuromuscular junction. Muscle Nerve 2006

Laryngeal Electromyographic findings in patients with vocal fold motion asymmetry

Vocal fold motion asymmetry (VFMA) is often attributed to vocal fold paresis or an anatomical variant. Although laryngeal electromyography (LEMG) may be used to evaluate patients with vocal fold paresis, electrodiagnostic findings in VFMA have not been well defined.

Analysis of laryngoscopic features in patients with unilateral vocal fold paresis.

The diagnosis of paresis in patients with vocal fold motion impairment remains a challenge. More than 27 clinical parameters have been cited that may signify paresis. We hypothesize that some features are more significant than others.