[Quality of life the goal of care for patients with ALS]. / God livskvalitet målet med vård och omhändertagande vid ALS - Enda sjukdomsmodifierande läkemedlet har begränsad effekt.

ALS is characterized by the degeneration of upper and lower motor neurons. In about 70% of patients with ALS the disease has an spinal onset, while about 30% of the patients have a bulbar onset.  Cognitive dysfunction and behavioral changes are seen in about 50% of the patients, and 15% develop frontotemporal dementia.  There is no single test that provides the ALS diagnosis. The diagnosis is based on clinical and electrophysiological signs, and the exclusion of other diseases. The only disease modulating treatment approved for ALS in Sweden is Riluzole, sadly only with limited effect. Other treatments are symptomatic and the goal is to help patients achieve the best possible quality of life through multidiciplinary ALS teams.

Dipyridamole and headache--a pilot study of initial dose titration.

Headache is reported by about one third of patients using dipyridamole (DP) after stroke or TIA. No study has systematically examined if initial dipyridamole titration may affect this headache. We therefore randomised patients to (1) standard aspirin and dipyridamole treatment b.i.d. for 2 weeks or (2) titration with aspirin only in the morning and aspirin and dipyridamole in the evening for 5 days, followed by 9 days of standard aspirin and dipyridamole treatment. Among 57 patients included for analysis, moderate to severe headache was reported by 28% in the standard treatment group and 25% in the titration group (n.s.). Headache for more than two consecutive days occurred in 24% and 11%, respectively. Rescue medication because of headache for more than 2 days was used by 14% and 0% in the respective groups. The cumulative number of days with headache was more than twice as high in the standard treatment group. The total numbers of other side effects were 25 and 11 in the two groups. The observed differences in this pilot study were not statistically significant, but nevertheless suggest that titration with an initially lower dose of dipyridamole may be considered to reduce headache and thereby increase compliance. A larger study is needed to clarify this with sufficient statistical power.

Diffusion-weighted MRI findings in patients with capsular warning syndrome.

BACKGROUND AND PURPOSE: The 'capsular warning syndrome' (CWS) of recurrent stereotyped episodes of motor or sensory dysfunction is clinically well recognized, and is associated with a high risk of imminent lacunar infarction with permanent deficits resembling those of CWS. However, the pathophysiology of CWS has not been well characterized. We report a clinicoanatomic correlation with MR imaging studies in the acute and chronic phases in patients with CWS. MATERIAL AND METHODS: Between April 1997 and March 2001, we prospectively studied 8 patients, mean age 73.3 years, presenting with 4-17 motor or sensorimotor transient ischemic attacks (TIAs; duration 2-90 min) up to 3 days after onset of the first episode. Four patients were free of symptoms between the attacks and had no residua, whereas 4 patients developed a pure motor or sensorimotor stroke within 1-3 days after symptom onset. Diffusion-weighted echoplanar MRI (DWI) and T(2)-weighted MRI studies were performed within 1 week after symptom onset and were repeated 1-2 months later. RESULTS: Seven of the 8 patients had an appropriate lesion on DWI in the acute phase. DWI abnormalities in the 3 patients with TIAs were 4-10 mm in diameter and confined to the lateral thalamus or medial globus pallidus without involving the internal capsule, whereas 4 patients who developed a stroke had abnormalities localized to the putamen extending to corona radiata (3 patients), or the pontomesencephalic junction (1 patient). All 6 patients who underwent follow-up MRI had an infarct on T(2)-weighted images corresponding to, but usually smaller than, the acute phase DWI abnormality. CONCLUSIONS: Small infarcts in the basal ganglia or the pons, close to central motor pathways, appear to be the primary lesion in CWS. The pathophysiology of CWS is complex, and may involve hemodynamic mechanisms in penetrating arterial territories, as well as molecular mechanisms, such as peri-infarct depolarizations affecting adjacent motor pathways.