Amyotrophic lateral sclerosis. Clinical analysis of 78 cases from Fortaleza (northeastern Brazil).

We report on the clinical characteristics of amyotrophic lateral sclerosis (ALS) in Fortaleza (Northeastern Brazil). For this, we analyzed retrospectively (from 1980 to 1999) 78 cases of ALS from the Service of Neurology of the University Hospital of Fortaleza diagnosed clinically and laboratorially (EMG, muscle biopsy, myelography, blood biochemistry, muscle enzymes and cranio-cervical X-ray). The results showed that they were mostly sporadic ALS (76/78), and they were divided into definite (n = 36), probable (n = 20), possible (n = 15) and suspected (n = 7), according to the level of diagnostic certainty. They were also subdivided into juvenile (n = 17), early-onset adult (n = 18), age-specific (n = 39) and late-onset (n = 4) groups. Clinically, they presented as initials symptoms, principally, asymmetrical (30/78) and symmetrical (24/78) weakness of extremities, besides bulbar signs, fasciculations, and atrophy. Curiously, pain as first symptom occurred in an expressive fashion (17/78). The predominant initial anatomic site, in this series, was the spinal cord, and mainly affecting the arms. As to the symptom accrual from region to region, this occurs more quickly in contiguous areas, and fasciculations are predominant when bulbar region was associated.

Nocturnal and respiratory disturbances in Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy).

Respiratory and sleep disturbances may be important causes of morbidity in Steele-Richardson-Olszewski syndrome but the frequency and character of nocturnal abnormalities remains uncertain. A prospective study of 11 patients with Steele-Richardson-Olszewski syndrome and age-matched control subjects was undertaken using clinical assessments, a structured sleep questionnaire, spirometry, static maximum inspiratory and expiratory pressures and nocturnal oximetry. The mean age of the Steele-Richardson-Olszewski syndrome patients was 63.2 (52-70) years and mean disease duration was 4.0 (2-6) years. There was moderate to severe motor disability in nine and mild to moderate dementia in eight. In the patients with Steele-Richardson-Olszewski syndrome the following abnormalities contributed to sleep disturbances significantly more frequently than in normal controls: depression, dysphagia, frequent nocturnal awakenings (usually associated with urinary frequency), immobility in bed, difficulty with transfers, impaired dressing and feeding. There was profound impairment of voluntary respiratory control whilst automatic and limbic control were well maintained. Nocturnal respiratory abnormalities were not present even in the most severely disabled. In Steele-Richardson-Olszewski syndrome sleep abnormalities are common; they relate to the cognitive, pseudobulbar and extrapyramidal disturbances and may therefore be amenable to symptomatic control.