Enhanced Bulbar Function in Amyotrophic Lateral Sclerosis: The Nuedexta Treatment Trial.

The goal of this randomized, blinded, crossover clinical trial was to determine whether Nuedexta (dextromethorphan and quinidine) enhanced speech, swallowing, and salivation in patients with ALS. Sixty patients with amyotrophic lateral sclerosis (ALS) received either Nuedexta or placebo for 28 to 30 days, followed by a 10 to 15-day washout period. Subsequently, patients were switched to the opposite treatment arm for the remaining days of the trial.

End-of-life decision making in 42 patients with amyotrophic lateral sclerosis.

Objective: To determine when end-of-life issues were discussed with patients afflicted with amyotrophic lateral sclerosis (ALS).

Methods: This was a retrospective analysis of ALS patients referred to the neuromuscular clinic at Georgetown University Hospital. Patients were seen by a pulmonologist and a neurologist at the initial diagnosis or referral, and every 2-3 months thereafter.

Collateral sprouting of human epidermal nerve fibers following intracutaneous axotomy.

Despite the clinical need, there are no therapeutic compounds available to promote peripheral nerve regeneration. In part, this may be due to a lack of sensitive measures of nerve growth. Here, we describe a novel approach of measuring collateral sprouting of epidermal nerve fibers (ENF) in human subjects and describe the effect of the neuroimmunophilin ligand timcodar dimesylate on collateral nerve sprouting.

Factors influencing nerve regeneration in a trial of timcodar dimesylate.

The authors assessed the ability of the neurophilin compound, timcodar dimesylate, to accelerate the return of epidermal nerve fiber density (ENFD) after a standardized nerve injury in a randomized double blind, placebo controlled trial. While there was no difference in the regeneration rate between the treatment and placebo arms, the baseline ENFD (p = 0.006), height (p = 0.

The time course of epidermal nerve fibre regeneration: studies in normal controls and in people with diabetes, with and without neuropathy.

We sought to develop and validate a standardized cutaneous nerve regeneration model and to define the rate of epidermal nerve fibre (ENF) regeneration first in healthy control subjects and then in neuropathic and neuropathy-free subjects with diabetes. Next, we assessed the effect of different factors on the rate of nerve fibre regeneration and investigated whether such an approach might offer insight into novel trial designs and outcome measures. All subjects had a standardized topical capsaicin dressing applied to the distal lateral thigh.

Intracranial hypertension in a dieting patient.

We report a case of encephalopathy with paranoid psychosis in association with intracranial hypertension. This occurred in a patient whose diet consisted almost solely of walnuts, ginseng tea, and vitamin A supplements. The patient was found to be severely iron- and vitamin B12-deficient.

Progressive motor neuron disease associated with electrical injury.

A patient in perfect health prior to electrical shock developed an ALS-like syndrome after the shock. Onset of the disease occurred in the limb through which the shock entered, and subsequently followed a course well-described in previous case reports. Possible mechanisms of disease are discussed.

Neuroretinitis in cat scratch disease.

We report a patient with neuroretinitis, whose associated lymphadenopathy, exposure to cats, and strongly positive cat scratch antigen skin tests suggested the diagnosis of cat scratch disease. Cat scratch disease should be added to the list of infectious agents believed to produce the clinical picture of neuroretinitis.

TRH-induced reflex changes in patients with ALS.

A trial of Thyrotropin Releasing Hormone (TRH) 5.0 mg/kg body weight subcutaneously every other day for two weeks produced transient increased tone in muscles, along with other (side-) effects in patients with Amyotrophic Lateral Sclerosis (ALS). One patient's extensor plantar transiently changed to a flexor plantar reflex after injection, probably due to disproportionate increase in tone of the calf muscles.

Treatment of amyotrophic lateral sclerosis with the TRH analog DN-1417.

Thyrotropin-releasing hormone has been reported to increase strength in patients with amyotrophic lateral sclerosis (ALS). DN-1417 is an analog of thyrotropin-releasing hormone, which has less endocrinologic activity, but more anterior horn cell stimulating effect (with no "autorefractory state"). However, 2 mg DN-1417, IM twice a day for 1 month in an open-label trial, produced no objective improvement of strength in nine patients with ALS.