No morphometric abnormality recurrent laryngeal nerve in spastic dysphonia.

Spastic dysphonia is a disorder of phonation that is usually markedly improved by surgical resection of one recurrent laryngeal nerve (RLN). In this study, biopsies of the RLN were obtained at surgery from nine patients with spastic dysphonia (disease group) and eight patients with laryngeal cancer (control group). The RLN was found to be composed of several nerve regions having characteristic fiber compositions. For the various nerve regions and for the whole nerve, we evaluated morphology, median fiber diameter, density, and size distribution of fibers. The morphologies of teased fibers were also evaluated. We found no significant differences between the nerves of the disease and control groups. Therefore, we were unable to verify previous reports of neuropathic abnormality of the RLN in spastic dysphonia.

Placebo-controlled trial of gabapentin in patients with amyotrophic lateral sclerosis. WALS Study Group. Western Amyotrophic Lateral Sclerosis Study Group.

We designed a phase II trial to evaluate the efficacy of gabapentin in slowing the rate of decline in muscle strength of patients with amyotrophic lateral sclerosis (ALS) and to assess safety and tolerability. Gabapentin (800 mg) or placebo was administered t.i.d. in a randomized, double-blinded, placebo-controlled, trial for 6 months. We enrolled 152 patients at eight sites in the United States. The primary outcome measure was the slope of the arm megascore, the average maximum voluntary isometric strength from eight arm muscles standardized against a reference ALS population. A secondary outcome measure was forced vital capacity. Slopes of arm megascores for patients on gabapentin were compared with slopes of those taking placebo using a two-way ANOVA. We observed a nonstatistically significant trend (p = 0.057-0.08) toward slower decline of arm strength in patients taking gabapentin compared with those taking placebo (mean difference 24%, median 37%). We observed no treatment effect on forced vital capacity. Gabapentin was well tolerated by patients with ALS. These results suggest that further studies of gabapentin in ALS are warranted.

A4V superoxide dismutase mutation in apparently sporadic ALS resembling neuralgic amyotrophy.

We report a case of apparently sporadic amyotrophic lateral sclerosis (ALS) in a young pregnant woman presenting subacutely with severe left shoulder pain followed by progressive weakness and wasting of the left arm, mimicking neuralgic amyotrophy. She was later found electrophysiologically to have widespread denervation involving more than just the arm and an alanine for valine substitution in codon 4 (A4V) in the gene for Cu/Zn superoxide dismutase 1 (SOD1). Her case illustrates that pain on initial presentation, though uncommon, does not exclude a diagnosis of ALS.

AAEM minimonograph #48: autonomic nervous system testing.

The autonomic nervous system maintains internal homeostasis by regulating cardiovascular, thermoregulatory, gastrointestinal, genitourinary, exocrine, and pupillary function. Testing and quantifying autonomic nervous system function is an important but difficult area of clinical neurophysiology. Tests of parasympathetic cardiovagal regulation include heart rate analysis during standing (the 30:15 ratio), heart rate variation with deep breathing, and the Valsalva ratio. Tests of sympathetic adrenergic vascular regulation include blood pressure analysis while standing, the Valsalva maneuver, sustained handgrip, mental stress, and cold water immersion. Tests of sympathetic cholinergic sudomotor function include the sympathetic skin response, quantitative sudomotor axon reflex test, sweat box testing, and quantification of sweat imprints. Pupil function is tested pharmacologically and with pupiilographic techniques. Tests of gastrointestinal and genitourinary function do not satisfactorily isolate autonomic regulation from their other functions. The available tests have various sensitivities and ease of administration. They are typically administered in a battery of multiple tests, which improves sensitivity and reliability, and allows probing of various autonomic functions.

Prostatic adenocarcinoma of ductal origin.

Adenocarcinomas that arise from primary or secondary prostatic ducts have distinctive histopathologic features. The age of patients, symptoms, findings on digital rectal examination and determinations of serum acid and alkaline phosphatase are similar to those of patients with acinic carcinomas. Carcinomas of secondary ducts may be less responsive to endocrine manipulation and of greater malignancy than carcinomas of primary ducts. The course and survival of patients with ductal carcinomas treated conservatively are poor.