Clinical features that distinguish PLS, upper motor neuron-dominant ALS, and typical ALS.

OBJECTIVE: To determine how clinical features at the first evaluation and in follow-up can be used to suggest a diagnostic outcome for patients with only upper motor neuron (UMN) signs at disease onset. METHODS: We reviewed the records of 34 patients (9 primary lateral sclerosis [PLS], 15 UMN-dominant amyotrophic lateral sclerosis [ALS], and 10 randomly selected control patients with ALS) seen in 1984-2007. Analysis of variance F tests for continuous variables and chi2 tests for categorical variables analyzed differences in baseline data among the diagnostic categories. Linear and generalized mixed effects models assessed the relation between examination data and diagnostic group over time. RESULTS: At first examination, the lowest score of the weakest muscle (p < 0.001), the site of onset (p = 0.041), and time to evaluation (p = 0.05) discriminated between eventual diagnostic group; patients with PLS were stronger, slower in progressing, and more likely to have limb onset than the other groups. Strength < or = 4 on any muscle was associated with the diagnosis of ALS (p = 0.0001), but not PLS. Across all visits, muscle strength (p = 0.003), ALS Functional Rating Scale score (p = 0.009), and vital capacity (p = 0.026) predicted group assignment. UMN-dominant and ALS groups had more weight loss (p = 0.004), even when controlled for dysphagia (p = 0.021) and muscle atrophy (p = 0.009), and patients with ALS were more likely to have hyporeflexia (p = 0.001). CONCLUSIONS: Features at baseline most suggestive of eventual lower motor neuron signs were focal muscle weakness or bulbar onset. Later, weight loss, reduced forced vital capacity, and limb weakness predicted lower motor neuron dysfunction. We suggest that patients with only upper motor neuron signs have periodic evaluations of strength, weight, forced vital capacity, Amyotrophic Lateral Sclerosis Functional Rating Scale score, and EMG, because a change in any can signal the imminent development of lower motor neuron signs.

The natural history of primary lateral sclerosis.

OBJECTIVE: To define the syndrome of primary lateral sclerosis (PLS) and disorders that contain features of both ALS and PLS, to determine the time beyond which PLS is less likely to become ALS clinically, and to determine the outcome of people with PLS and those who develop lower motor neuron (LMN) signs. METHODS: The authors reviewed the records of all 39 patients initially diagnosed with PLS in 1984 to 2004. Diagnostic subgroups were defined based on clinical features. The authors used Kaplan-Meier methods to estimate the time to diagnosis, linear regression analyses to assess function, and a Cox proportional hazard model to assess survival in subgroups. RESULTS: Of the 39 patients, 29 had only upper motor neuron (UMN) signs on initial evaluation. Thirteen of the 29 were later classified as having UMN-dominant ALS (UMN-D) because they acquired evidence of denervation by EMG (3.17 years) or examination (3.67 years). Sixteen of the 29 patients, classified as clinically pure PLS, retained only UMN signs and a normal EMG (mean follow-up 8.7 years). Ten patients who met criteria for ALS at the initial visit were used as controls. The UMN-dominant ALS group had lower functional scores (p = 0.033) than the PLS group, and similar scores to those with ALS. Survival was longer in both the PLS group (p = 0.027) and the UMN-D group (p = 0.067) than the ALS group. CONCLUSIONS: Clinically pure PLS can be defined by isolated UMN signs 4 years after symptom onset, and is a syndrome of slow progression with high levels of function. Prior to the fourth year, the diagnosis of PLS cannot be made with certainty because many patients develop LMN signs. UMN-dominant ALS, defined by predominantly UMN disease with minor LMN signs, has disability similar to ALS, but slower progression.

Employee assistance: a business and community hospital partnership.

Employers are now providing a variety of healthcare services to combat health problems that affect productivity. These employee assistance programs are designed to improve work performance by providing an approach to the prevention, diagnosis, and treatment of such illnesses as alcoholism, drug dependence, mental illness and family, martial, financial, and legal difficulties common to all worksites. This article examines the concept, incentives, structure, obstacles, marketing, and cost containment factors that are part of the development of this new partnership between businesses and community hospitals.

Planning and developing a prehospital mobile intensive care system in an urban setting.

A suggested model for the development of an urban based prehospital emergency care system is described. Factors considered in the planning and development include: 1) demand for services, projected and actual; 2) analysis of costs; 3) design and maintenance of the delivery system; and 4) establishment of the evaluation mechanisms. Over one year's experience and 1,144 mobile intensive care unit (MICU) calls in a densely populated urban setting with over 500,000 persons are reported. During the peak 8-hour period, predetermined dispatch categories were employed to activate one MICU operating in conjunction with three conventional ambulances. This partial conversion imparted MICU capability to the entire system at an 11 per cent increase in the ambulance budget. MICU calls averaged 4.5 per 8-hour peak shift and took 45 minutes each.

Pregnancy in the unwed White South African.

A sociological and obstetric analysis of 130 unwed Afrikaans- and English-speaking mothers showed that 78% of English-speaking and 61% of Afrikaans-speaking mothers preferred to keep their babies. More than 80% of women in both language groups did not use contraceptives. There were no statistically significant differences between the unwed mothers and a married control group as regards antenatal anaemia, toxaemia of pregnancy, antepartum haemorrhage and breech presentation. Labour lasted longer than 18 hours in 8.6% of English-speaking unwed mothers but in none of the Afrikaans group. English-speaking mothers who gave up their babies for adoption had a higher incidence of prolonged labour, toxaemia, forceps delivery and premature birth.