Gastric and enteric involvement in progressive systemic sclerosis.

Progressive systemic sclerosis (PSS) is a chronic multisystem disease characterized by excess deposition of connective tissue in skin and internal organs, associated with microvasculature changes and immunologic abnormalities. Involvement of the gastrointestinal tract may occur in 2 stages, a neuropathic disorder followed by a myopathy. Gastric emptying is delayed in 10% to 75% of patients and correlates with symptoms of early satiety, bloating, and emesis. Compliance of the fundus is increased although perception of fullness is normal. Myoelectric abnormalities have been found in some studies. Treatments include metoclopramide, cisapride, and erythromycin. Bleeding from telangiectasias and watermelon stomach is treated endoscopically. Small bowel involvement in PSS occurs in 17% to 57% of patients. The migrating motor complexes are reduced or absent, predisposing to bacterial overgrowth. Malabsorption may also be due to pancreatic insufficiency. Barium enemas demonstrate pancolonic involvement in 10% to 50% of patients with PSS. Wide-mouthed diverticuli, involving all layers of the intestinal wall, are characteristic. Pseudoobstruction may respond to octreotide or prucalopride therapy. Complications include pneumatosis cystoides intestinalis, stercoral ulcerations, and perforation. Fecal incontinence may be due to dysfunction of the internal anal sphincter, a smooth muscle responsible for most of the resting anal sphincter pressure. Anal manometry may show a reduction or loss of the rectoanal inhibitory reflex. Treatments include biofeedback, sacral nerve stimulation, and surgery. PSS involves the gastrointestinal tract from the mouth to the anus. Studies are needed to define effective treatments in these diseases, which cause great morbidity.

CSF orexin levels of Parkinson's disease, dementia with Lewy bodies, progressive supranuclear palsy and corticobasal degeneration.

Excessive daytime sleepiness has been widely accepted as a common problem not only in Parkinson's disease (PD) but also in other related disorders. Lowered excretion of orexin A (hypocretin 1) into the cerebrospinal fluid (CSF) is known to play a pathological role in narcolepsy and secondary hypersomnia due to hypothalamic dysfunction. Although the levels of CSF orexin in PD have been previously examined, the results have been controversial, and no systematic investigation of CSF orexin excretion has been conducted on PD related disorders. In this study, orexin was measured in CSF collected by lumbar puncture in 62 patients with PD, 13 patients with dementia with Lewy bodies (DLB), 16 patients with progressive supranuclear palsy (PSP), and 7 patients with corticobasal degeneration (CBD). Levels of CSF orexin (mean+/-SD pg/ml) were 302+/-38 in PD, 297+/-48 in DLB, 258+/-37 in PSP, 246+/-90 in CBD. The occurrence of low orexin levels (

[Does garlic influence rheologic properties and blood flow in progressive systemic sclerosis?]. / Beeinflusst Knoblauch die Blutfliesseigenschaften und die Durchblutung bei progressiver systemischer Sklerose?

BACKGROUND: According to traditional European naturopathy garlic is an agent that increases perfusion. In studies with healthy subjects and in-vitro research garlic has shown influences on erythrocyte and thrombocyte aggregation as well as on vasoregulation. However, data on its effects in clinical populations are still lacking. Garlic may be useful for systemic sclerosis which is characterised by impaired perfusion that often cannot sufficiently be influenced by standard treatment. We investigated if dried garlic powder can influence rheologic properties and vasomotor function in systemic sclerosis. SUBJECTS AND METHODS: During a randomised, double blind pilot study, 20 female inpatients with systemic sclerosis received a 7 day add-on therapy with either 900 mg dried garlic powder or placebo. Rheologic properties (erythrocyte aggregation, ADP-induced thromboycyte aggregation, plasma viscosity, fibrinogenous plasma level, blood sedimentation rate) were assessed initially as well as after 1 and 7 days of treatment. Vasomotor function was evaluated using near-infrared red photoplethysmography, a new diagnostic tool to assess microcirculation. Furthermore, acral skin temperature was measured. RESULTS: After 7 days, only the verum treatment had induced a significant reduction of ADP-induced thrombocyte aggregation and a decrease in erythrocyte aggregation. Results showed no significant effects on vasomotor function, but an immediate effect of garlic on acral skin temperature. DISCUSSION: According to the 'Qualitatenlehre' of traditional European naturopathy, garlic is classified as a 'heating agent'. Our results suggest that the improvement of rheologic properties could be a possible biological correlate for this. Although further research is required, we conclude garlic could be a rational add-on therapy in the 'Kaltekrankheit' ('cold disease') of systemic sclerosis.