Journal of neurology
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Journal of neurology · Oct 2011
Clinical TrialAsymptomatic small fiber neuropathy in diabetes mellitus: investigations with intraepidermal nerve fiber density, quantitative sensory testing and laser-evoked potentials.
This study aimed at evaluating the performance of a battery of morphological and functional tests for the assessment of small nerve fiber loss in asymptomatic diabetic neuropathy (DNP). Patients diagnosed for ≥10 years with type 1 (n = 10) or type 2 (n = 13) diabetes mellitus (DM) without conventional symptoms or signs of DNP were recruited and compared with healthy controls (n = 18) and patients with overt DNP (n = 5). Intraepidermal nerve fiber density (IENFd) was measured with PGP9.5 immunostaining on punch skin biopsies performed at the distal leg. ⋯ Defining a critical small fiber loss as a reduction of IENFd ≤-2 z scores of healthy controls, this state prevailed in type 2 (30%) over type 1 DM (10%) patients despite similar disease duration and current glycemic control. LEPs with the small laser beam performed best in terms of sensitivity (91%), specificity (83%) and area-under-the ROC curve (0.924). Although this performance was not statically different from that of warm and cold detection threshold, LEPs offer an advantage over QST given that they bypass the subjective report and are therefore unbiased by perceptual factors.
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Journal of neurology · Oct 2011
Quality of life of ALS and LIS patients with and without invasive mechanical ventilation.
There are very few studies where quality of life (QOL) is assessed in patients with complete physical and functional disability and dependence to invasive mechanical ventilation (IV). We compared QOL of amyotrophic lateral sclerosis (ALS) and locked-in-syndrome (LIS) patients with invasive mechanical ventilation to ALS and LIS patients without mechanical invasive ventilation. Thirty-four patients, 27 with ALS and seven with LIS (vascular or tumoral aetiology) were included in the study. ⋯ Comparison between IV group and LIS without invasive mechanical ventilation revealed no significant difference for SF36 and McGILL QOL scores. QOL was not significantly different between the IV and not invasively ventilated patients, but ALSFRS was significantly lower in the IV group, and comparison of QOL scores between non-ventilated LIS patients who had the same score of dependence that invasively ventilated patients did not show any difference. Invasive mechanical ventilation for patients who accept tracheotomy allows life prolongation and their QOL is not affected; medical teams should be aware of that.
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Journal of neurology · Oct 2011
High disease impact of myotonic dystrophy type 2 on physical and mental functioning.
The aim of the study was to investigate health status in patients with myotonic dystrophy type 2 (DM2) and determine its relationship to pain and fatigue. Data on health status (SF-36), pain (MPQ) and fatigue (CIS-fatigue) were collected for the Dutch DM2 population (n = 32). Results were compared with those of sex- and age-matched adult-onset myotonic dystrophy type 1 (DM1) patients. ⋯ From the perspective of health-related quality of life, DM2 should not be considered a benign disease. Management of DM2 patients should include screening for pain and fatigue. Symptomatic treatment of pain and fatigue may decrease disease impact and help improve health status in DM2, even if the disease itself cannot be treated.