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Botulinum toxin (BTA) is a safe and effective therapeutic tool for many neurological conditions but few studies have investigated its real cost in neurological practice. We evaluated the daily cost of botulinum toxin type A (BTA) treatment through a cost effectiveness analysis alongside a prospective study of BTA injections at a French University Hospital over a 2-year follow-up period. The data of 3,108 BTA injections performed in 870 adult patients presenting with dystonia, facial hemispasm, or spasticity were entered in the database. Patients were questioned at each visit about the subjective effectiveness of the previous injection. The daily cost of BTA treatment was calculated as the ratio of each session's cost (including all additional costs) to the duration of subjective efficacy. The subjective rating of efficacy duration was 17.3 ± 7.7 weeks for facial hemispasm, 15.4 ± 7.7 for blepharospasm, 14.3 ± 6.7 for cervical dystonia, 14.5 ± 7.8 and 14.1 ± 7.4 weeks for upper and lower limb spasticity, respectively. The daily cost of BTA injections was 0.57 ± 0.20 € for facial hemispasm, 0.95 ± 0.30 € for blepharospasm, 2.85 ± 0.86 € for cervical dystonia, 3.38 ± 1.49 € and 3.62 ± 1.81 € for upper and lower limb spasticity, respectively. When associated costs were considered, the daily cost of BTA injections was clearly increased (45-93%) in limb spasticity or rigidity but remained only modestly increased (15-37%) in other indications. These results obtained in a large cohort of patients show that BTA treatment has a low daily cost for a long-lasting effect, with a daily cost/benefit ratio that greatly depends on the indications. [ABSTRACT FROM AUTHOR]

A 73-year-old man was investigated for a peripheral neuropathy which occurred in the course of a Waldenström's macroglobulinemia. Serum immuno-fixation electrophoresis demonstrated two IgM monoclonal gammopathies of the kappa and lambda chain isotypes, and one had the physical characteristics of cryoglobulin. Immunoblot studies on the patient's serum revealed antibodies which reacted with peripheral nervous system proteins of different molecular weights including the myelin-associated glycoprotein (MAG). An immunofluorescence study of a superficial peroneal nerve biopsy revealed not only a binding of IgM and kappa light chain on several myelin sheaths but also the presence of IgM and kappa light chain deposits in the endoneurium. On electron microscopic examination, numerous fibres presented a widely spaced myelin and the endoneurial deposits had the ultrastructure of cryoglobulin. This is the first case presenting features of widely spaced myelin related to serum anti-MAG activity associated with monoclonal cryoglobulin deposits in the endoneurium. [ABSTRACT FROM AUTHOR]

We describe a peculiar form of late onset paroxysmal cerebellar ataxia including clinical features similar to episodic ataxia type 2 (EA2) but unresponsive to acetazolamide. Four unrelated patients were clinically investigated. Neuropathological examiniation was performed in one patient and molecular anlaysis in all four. All 47 exons of CACNA1A were screened by a combination of single-strand conformer polymorphism and sequencing analysis in three patients. In addition, the length of the CAG repeat was determined in all four patients. The four patients were in their 60s at the onset of the disease, which was characterized by cerebellar ataxia attacks lasting from a few minutes to 1–2 h and occurring mainly in the morning. In the interictal period a nystagmus was present together with a slowly progressive cerebellar ataxia over the years. The neuropathological examination disclosed a dramatic loss of Purkinje cells mainly in the vermis. Moreover, certain cerebellar granular neurons had a strong cytoplasmic, staining at immunopathological examination with an anti-tau protein serum. Search for truncating mutations or CAG repeat expansion in CACNA1A was negative. This lateonset paroxysmal cerebellar ataxia with neutropathological lesions restricted to Purkinje cells and with negative results both for truncating mutations and CAG expansion in the CACNA1A gene represents a new entity. Further studies are needed to delineate the underlying process. [ABSTRACT FROM AUTHOR]

The possible occurrence of chronic inflammatory demyelinating polyneuropathy (CIDP) in association with an identified dysglobulinemic status is recognized and a causal relationship between the two has been suggested. We had the opportunity to study 18 patients presenting with CIDP and dysglobulinemia. This was an IgG monoclonal gammopathy (IgG MG) in 8 cases, an IgM monoclonal gammopathy (IgM MG) in 8, an IgG-IgM biclonal gammopathy in 1 case and an IgM monoclonal cryoglobulinemia in another. A peripheral nerve biopsy specimen was available for all patients and the morphological findings in these specimens in the cases of CIDP with IgG MG or cryoglobulin did not differ from those without, whereas characteristic features were observed in the cases of CIDP with IgM MG and anti-myelin associated glycoprotein activity. [ABSTRACT FROM AUTHOR]

Following intravenous administration, the uptake of colloidal drug carriers by cells of the mononuclear phagocyte system, mainly the Kuppfer cells, may concentrate an encapsulated drug close to the liver parenchymal cells and facilitate its biliary excretion and enterohepatic circulation. To test this hypothesis indomethacin was administered (10 mg/kg) in four groups of 10 rabbits each by intravenous infusion at a constant rate over 2 hr, either in its free form (aqueous solution) or as nanocapsules prepared from preformed poly(DL-lactide). Unchanged drug was assayed in plasma of the two control (sham-operated) groups and in both plasma and bile of the two bile-cannulated groups. Pharmacokinetic analysis led to the conclusion that the uptake of nanocapsules by liver macrophages reduces the concentration of the drug by enhancing its total clearance. This enhancement was due to an increase in biliary clearance, as a result of parallel increases in bile concentration and biliary excretion of the drug. It was also demonstrated that nanocapsules enhance the enterohepatic circulation of indomethacin. [ABSTRACT FROM AUTHOR]

We studied the efficacy of endoscopic injection of Botulinum A toxin (150 I.U. Dysport®) in the treatment of detrusor-sphincter dyssynergia in 17 patients with spinal cord disease. One month after the injection, the postvoiding residual urine volume (-176 ml, P<0.001), the bladder pressure on voiding (-19 cm water, P<0.01), and the urethral pressure (-24 cm water, P<0.001) were significantly decreased. The modality of voiding was improved in 10 patients (ie micturition by suprapubic tapping was easier to induce, discontinuation of indwelling catheter use, or decrease in frequency of intermittent catheterizations). The tolerance of the treatment was excellent. The therapeutic effect lasted 2 to 3 months on the average. The low doses used in this study probably explain in part why the treatment sometimes failed. Botulinum A toxin could become an alternative treatment for detrusor-sphincter dyssynergia in certain patients, notably in those who are refractory to sphincterotomy or in patients, such as those who are tetraplegic, and who are incapable of performing intermittent self-catheterization. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented titled "Long-term improvement of paroxysmal dystonic choreathetosis with acetazolamide," by Veronique Michel and others in the Journal of Neurology vol. 253, 2006 issue.